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MCQ Nelson 20
MCQ Nelson 20
MCQ Nelson 20
Pediatrics
QUESTIONS
AHMEDTAWFI
cause
common
1.TheMOST ofunder-5 worldis
Indeveloping
mortality
A. diarrheaidisease
8 pneumonia
C malaria
D. measles
E. neonataldisease
5. A full term neonate with Down syndrome and esophageal atresia admitted in
Pediatricintensive care unit (PiCU). The parents want no interference" approach.
0f the following,the BESTresponseis to
A. act accordingto parents weli
3. arrangethe operation with the surgeon
C. Workfor the patient best interest
D. givesupportivecareonly(DANR)
E. wait for a clear legislation
6.Youareresident called
inPediatrics, to attend
atmidnight of
delivery baby
expected
ashadbeenreportedIn antenatal
andoligohydromnias
withmultiplemalformations
scan, mother in stage I labor so far.
Of the following, your BESTplan of action is to
A. counsel the parentsthat you will go for (DNAR)status
B. start your usual resuscitationwaiting for your senior
C. explain that the result of antenatal scan is final
D. call your consultant or senior resident
E. apply palliative care for the coming neonate
7. A 7-yearold boy presented to the out-patient department with fever and cough, you
have diagnosed him as a case of pneumonia and decided to admit and give parental
therapy, the child refused.
Of the following, the BESTresponse is to
A. explain to father about dangers of omitting treatment
B. try to explain the risk to the child
C. give the chance for oral antibiotics and accept the child decision
D. inform your consultant
E. inform hospital ethical committee
8. You are treating acutely ill 6-year-old child with ascending paralysis, he eventually
needs mechanical ventilation. Now you are in a situation of
using the last bed in
respiratory care unit (RCU).
Of the following, the BEST approach is to
A. try to convince the parents that, there is no use of ventilation
keep the bed for more treatable condition
try bedside ventilation
11. After the first year of life, the MOST common cause of death in children is mainly
none-accidental injuries
burn
W909? drowning
accidents
war
13. Around 44,000-98,000 patients die in US. hospitals each year because of
preventable medical errors, these figures can be reduced if adapting an emerging new
science of Quality improvement (QI).
The TRUEfigure of children receive recommended health care in USAis about
A. 16%
26%
PD!
36%
46%
80%
14. Patient culture can change the doctor treatment options and even the way of
behavior with the patient.
All the following culture values are expected if he is dealing with Muslim patients
EXCEPT
A. fasting during Ramadan month
3- modestyand female body cover .
C. forbiddance of touch and shake hands of opposutesex
D. routine postmortem exam
E. patriarchal way of decision making
The Field of Pediatrics
ANSWERS
HMED TAWFIQ,
1 (E). Causes of under-5 mortality differ markedly between developed and developing
nations. In developing countries, 66% of all deaths resulted from infectious and
parasmc diseases. Among the 42 countries haying 90% of childhood deaths, diarrheal
disease accounted for 22% of deaths, pneumonia 21%, malaria 9%, AIDS 3%, and
measles 1%. Neonatal causes contributed to 33% of the total death but 24% of deaths
are caused by severe infections and 7% by tetanus.
2 (E). Bacterial sepsis constitutes only 2%. Other distracters are arranged from common
to least common.
3 (C). In severe form of mental retardation the souoeconomic factor is not effective
parameter while in mild forms it plays a role.
4 (B). DNAR status is a statement issued by medical ethical group (which is a group of
consultants) regarding certain untreatable medical illnesses, it means do not attempt
active resusCItation, so all supportive care, medications, and 02 are allowed, adrenaline
is a medication used in active resusatation.
S (C). Pediatrician is a child advocate; he must take deCISIon always to the "best
interest" of the child that what is called beneficent paternalism in pediatrics which is
the obligation to act in a younger childs best interest that takes moral precedence over
the Wishes of the childs parent(s), so in such a scenario arrangement with the surgeon
to do the operation and calling the local hospital committee to protect the child is the
best choice but this action is still need legislation in the third world. In a such Situation
the Pediatrician must explain the benefit of treatment to the parents (baby best
interest) trying best efforts to save the baby keeping in mind Down syndrome babies
are not DNAR status.
6 (D). In such situation when you have time (mother still in stage I labor), the best
response is to call your direct senior resident or consultant then explain the result of
antenatal scan trying to put the parents in the expected situat on. Starting the usual
resuscitation is the best chaice when there is no time and the baby arrived. The
decismn of (DNAR) should be made by the ethical hospital committee or supervising
consultant and should be powered by national legislations.
7.(C). In older children and in noncritical situation the prinCiple of the "child
assentwhich means the child voice must be heard should be applied. In this scenario,
the situation is Mild and the child non acceptance of inyectable medication should be
respected. it is still in critical situation or more acute illness the child best interest
should be sought and it may be against his well.
8 (D). It is clear in this scenario that the child suffers potentially treatable condition. In
this Situation use the best resources and the best care. The choices at (A84?) are
definitely non ethical Other choicesare optional when there is no bed ls available in
RCUtaking the best interest for the patient in consideration.
9.(A). In such difficult situation (chronic disablingillnesses)and the child In vegetative
state; use the approach from B to E steps and allow the final decision to the parents
especially if you are not covered with clear legislations.
10 (C). This is a well child visit; treatment plansare not included here.
1140). The most common cause of death in children beyond the first few months of
life is injurieswhich includesroad traffic accidentsand other none-intentional injuries,
with up to 5060% of total causesof death.
12 (C). The most successfulinjury preventionstrategiesgenerally are those involving
changes in product design. Such as making more secure cars, more suitable car seats
...etc.
13 lo).
14 (D). All the above valuesare expectedfrom Muslimculture,except submittingthe
dead body for routine postmortem exam. The value (E) means the way of decision
making is usually left noolder person in the family.
Growth, Development, and Behavior
QUESTIONS
HMEQ TAWFl
1. In embryonic period, all the following are true EXCEPT
formation of blastocyst by 8 days
formation of endoderm and ectoderm by 2 weeks
ofa babYOeddoll's-eye
2week-fullterm Ofher
movementbaby
eyes.
6Amother
' is
action to
0f by
a
her
next
eMOSTpropriate
that,
inforfhing reaction
isanormal
this
ti:$22322?
toan ophthalmologist
thebaby
8. refer
ofperinatal
history
adetailed
C. takes period
D. orderbrainultrasound
E. order brain MRI
achieve
theinfant
7,Theageatwhich withbobbing
earlyheadcontrol motion
when
pulled to sit is
A. 2mo
B. 3mo
C. 4mo
D. Smo
E. 6mo
9. By the age of 7 months the infant is able to do all the following EXCEPT
A. transfer object from hand to hand
B. bouncesactively
C. cruises
D. grasp usesradial palm
E. roll over
91.00.?
visual, gross,and fine motor coordination
able to link actions to solve problems
symbolic thought
A. 24 mo
8. 30 mo
C. 36 mo
0. 42 mo
E. 48 mo
15. The child who continue to search for a hidden subject, has achieved the
developmental age of
A. 7mo
B. 9mo
C. 11 mo
D. 13 mo
E. 15 mo
16. The child who's able to standsmomentarily on 1 foot, makes tower of 10 cubes;
imitates construction of bridge of 3 cubes; copies circle and imitates cross is
A. 24 mo old
8. 30 mo old
C. 36 mo old
D. 42 mo old
E. 48 mo old
a4 who
child has and ofinitial
repetitions
pauses 5°Unds
17. A th
mor toe year-old -
department.
visitedoutpatients is
' advice
Of h MOSTappropriate 5%
about
affecting
phenomenon of .
children
preschool
/1:fghlgvivslggnorriial
as80%
foraction ofaffected recover
children bytheir
own
isnoneed
B there withspeaking
toreduce
C.tries associated
pressures
0. I'llreferhimtoENT
specialistevaluation
forfurther
E. I'll referhimto speechtherapist
factsaretrueregarding
18 These stageof preschool
thedevelopmental children
EXCEPT
is achievedby 3 yearsof age
A. handedness
bladdercontrol
laterthangirlsinachieving
B. boysareusually
C. knowing gender by 4 years
D. egocentric thinking
E. musturbation
10
A. 1%
B. 5%
C. 10%
D. 30%
E. 50%
22. A medical student asked you during the morning round about his observation of
infants cry in response to the cry of another infant.
of the following, the MOST appropriate answer is that it represent
A. an early sign of empathy development
3. a sign of good hearing
C. a startle reflex to a loud sound
D. an early sign of fear development
E. an early sign of autistic behavior
23. Between 2-6 months of life, the infant start to achieve a regular sleepwake cycles.
All the following are true about infant sleep during this period EXCEPT
A. total sleep hours are about 14-16 hr/24 hr
B. sleeps about 9-10 hr concentrated at night
C. sleeps 2 naps/day
D. sleep electroencephalogram shows the mature pattern
E. the sleep cycle time is similar to that of adults
24. A highly careful mother of a 10-month-old baby boy complaining that her baby has
inadequate weight gain. She explained to you how she is so strict and so careful to feed
her baby by spoon, but he was always refusing.
0f the following, the LEASThelpful advice is to
A. respect infant independence
8. offer softer diet
C. use 2-spoons (1 for the child and 1 for the parent)
D. use finger foods
E. use high chair with tray table
25. The child who helps to undress; puts 3 words together (subject, verb, object); and
handles spoon well, his/her MOSTappropriate age is around
A. 15 mo
B. 18 mo
C. 24 mo
D. 30 mo
E. 48 mo
11
ChdIIIIII, I I I I e
who
26"laand a S le ("I
gallles
Silll e
pala- ' ps
in
w
dlessing
A. 15 mo
8. 18 mo
C. 24mo
D. 30 mo
E. 48 mo
'5 to
ableimitates ofgate0f5cubes;
construction draws
aman
27'TheCthWho of2lines,
longer
identifies MOST
his/her appropriate
2-4 5
beside
with parts and
head
ageisaround
developmental
A. 24 mo
B. 30 mo
C. 48 mo
D. 54 mo
E. 60 mo
28.The usually
birthweight bytheageof
quadruples
A. 1.5 yr
8. 2 yr
C. 2.5 yr
D. 3 yr
E. 3.5 yr
thephysical
29.Regarding of preschool
growth are
(3-5yr);allthefollowing
children
true EXCEPT
A. 4-5 kgweightincrement/yr
B. 7-8 cm height increment/yr
C. head will grow only an additional 5-6 cm up t018 yr
0. all 20 primary teeth have erupted by 3 yr
E. average wt 20 kg and height 40 inch by 4 yr
30'Regarding
thephysical ofmiddle
growth are
(6-11yr);allthefollowing
childhood
true EXCEPT
A. 3-3.5 kg wt increment/yr
B. 6-7 cm height increment/yr
C. brainmyelinizationstopsby 8 yr
D. firstdeciduoustooth fallsby 6 yr
6. riskfor future obesity
31.Thefirstpermanenttoothto
eruptis
A. centralincisorat 6 yr
12
8.molar at 6 yr
C. premolar at 6-7 yr
0. lower canine at 6-7 yr
E. upper canine at 6-7 yr
33. The MOST common cause of sleepingdifficulty in the first 2 months of life is
A. gastro-esophageal reflux
B. formula intolerance
C. colic
D. developmentally sleeping behavior
E. recurrent self resolving intussusceptions
34. A mother of 4-month-old baby boy complaining that the baby never goes to sleep
unless he is rocked for quite long time. She needs to repeat the same issue whenever
he is awake at night. She was surprised as no one of her previous babies had the similar
behavior. She feels tired because of insufficient sleep.
Of the following, the MOST common cause of this baby problem is
A. an early signs of ADHD
B. behavioral insomnia of childhood, sleep-onset association
C. primary sleep disorder
D. restless legs syndrome
E. sleep terrors
36. All the following are included in basic principlesof healthy sleep EXCEPT
A. set bedtime routine
8. avoid stimulating activities as playing computer games
13
sure
C. makes child
your spendstimeoutside
every
day
meal
aheavy
0. gives an
within or
hour 2ofbedtime
setoutofyour
thetelevision
E. keeps bedroom
childs
arecompatible
37.Allthefollowing of obstructive
withthedeftnltion sleepapnea
EXCEPT
A. repeated ofprolonged
episodes upper obstruction
airway
8. apnea
C, 230% reduction in airflow
D. 2 30% 02 desaturation
E. disrupted sleep
39. Personswith Down syndrome are at particularly high risk for obstructive sleep
apnea with up to 70% prevalence.
All the following are considered as risk factors EXCEPT
A. peculiarfacial anatomy
8. hypotonia
C. developmentaldelay
0. central adiposrty
E. hypothyroidism
14
Growth, Development, and Behavior
ANSWERS
HMED TAWFI
15
17 (c). Developmental dysfluencyor stuttering it includes a pauses and repetitions of
initial sounds.Stress or excrtementexacerbates these difficulties, which generally
resolve on their own. Although 5% of preschool children will stutter, it Will resolve In
80% of those children by age 8 yr. Children With stuttering should only be referred for
evaluation if it is severe, persistent, or assocratedwrth anxiety, or if parental concern is
elicited. The best response is to give guudance to parents to reduce pressures
assouated With speaking. Other statements can be used for reassurance and for
explaining the natural history of the problem.
18((2). Bowel and bladder control emerge during this period, With readiness" for
toileting havrng large indiVIdual and cultural variation. Bed-wetting is normal up to age
4 yr In girls and age 5 yr in boys. Thinking in this period characterized by magical
thinking (the child might believe that people cause it to rain by carrying umbrellas, that
the sun goes down because it is tired), and egocentrism which refers to a childs
inability to take anothefs paint of view. Gender identification usually achieved by 3
years of age. CuriOSity about genitals and adult sexual organs is normal, as is
masturbation. But excessive masturbation interfering wrth normal activity, acting out
sexual intercourse, extreme modesty, or mimicry of adult seductive behaVior all
suggests the p055ibility of sexual abuse or inappropriate exposure.
19 (B). Clas5ical temper tantrums of children usually result from fear, overtiredness,
inconsistent expectations, or physical discomfort. Tantrums normally appear toward
the end of the lst yr of life and peak in prevalence between 2 and 4 years of age.
Tantrums lasting more than 15 min or regularly occurring more than 3 times/day may
reflect underlying medical, emotional, or soaal problems. In the scenario the episodes
takes long time to resolve and the mother paints to problem in soaal development.
Here we need to investigate the developmental domains and to do systemic
examrnationto checkoutfor any developmental/phy5icalproblem before referring the
child to pediatric psychiatristwhich the next most appropriate chorce.
20 (C). Prolongcryingepisodesl5 present in about 20% of infants younger than 2 mo of
age Crying(longer than 3 hr/day, longer than 3 days/wk lasting longer than 1 wk) and
more often crying/fussmesspersistinglonger than 3-5 mo might be assoaated wrth;
child abuse; behavroral problems when an older child (anXIety, aggressmn,
hyperactiwty); decreased duration of breast-feedingand postnatal depression
(uncertainWhichis the causeor effect). Most infantsWith crying/fussinessdo not have
gastroesophagealreflux,lactoseintolerance,or cow-milkproteinallergy.
21 (a).
22 (A).
23 (E). The sleepcycleremainsshorterthan in adults(50 60 min vs approximately90
mm). A; a result,infantswho arousesfrequentlyduring the night. consideredas
behaVioral
Sleepproblem.
24(3). Poorweight83" it this age often reflectsa strugglebetweenan infant's
emerging andParents
independence control Situation.
ofthefeeding
25 (Cl-
16
26.(D).
27.(C).
28.(C).
29.(A). 2kg/yr.
30.(C). Brain myelinization continues into adolescence, with peak gray matter at 12-14
yr.
31(3). The first permanent molars usually erupt between ages 6 and 7 years. For that
reason, they often are called the six-year molars. They are among the extra"
permanent teeth in that they don't replace an existing primary tooth. These important
teeth sometimes are mistaken for primary teeth. The six-year molars also help
determine the shape of the lower face and affect the position and health of other
permanent teeth. The first deciduous teeth to fall are central incisors.
32.(A). Melatonin is secreted from penile body. Two sleep cycles; the slow-wave sleep
(SWS) (i.e., n3, delta, or deep sleep) appears to be the most restorative form of sleep; it
is entered into relatively quickly after sleep onset, it is preserved in the face of reduced
total sleep time, and it increases (rebounds) after a night of restricted sleep. Rapid eye
movement (REM) sleep (stage r or dream sleep) appears to be involved in (1)
completing vital cognitive functions, such as the consolidation of memory; (2)
promoting the plasticity of the central nervous system (CNS); and (3) protecting the
brain from injury.
33.(D). Most sleep issues that are perceived as problematic at this stage represent a
discrepancy between parental expectations and developmentally appropriate sleep
behaviors. Newborns that are noted by parents to be extremely fussy and persistently
difficult to console are more likely to have one of the mentioned medical issues.
34(8). In this situation, the child learns to fall asleep only under certain conditions or
associations, which typically require parental presence, such as being rocked or fed,
and does not develop the ability to self-soothe. in babies, gradually weaning the child
from dependence, and in older children introduction of more appropriate sleep
associations that will be readily available to the child during the night (transitional
objects, such as a blanket or toy), in addition to positive reinforcement (i.e., stickers for
remaining in bed), is often beneficial.
35.(D). Is often the result of parental difficulties in setting limits and managing
behaviour in general, and the inability or unwillingness to set consistent bedtime rules
and enforce a regular bedtime in particular.
36.(D). Other advices also include; dont send your child to bed hungry. A light snack
(such as milk and cookies) before bed is a good idea. Heavy meals within an hour or 2
of bedtime, however, may interfere with sleep. Avoid products containing caffeine for
at least several hours before bedtime. These Include caffeinated sodas, coffee, tea, and
chocolate. Keep your childs bedroom quiet and dark. A low-level night light is
acceptable for children who find completely dark rooms frightening. Keep your childs
bedroom at a comfortable temperature during the night (<24'C). Dont use your child's
17
Keepthe televisionset out of yourchild's
bedroomfor time-outor punishment.
bedroom.
37.10). 2 3% 02 desaturatlon.
38 (A).
39.03. Macroglosia.
40(A). NarcolepsyIs a recurrent episode of excessive daytime sleepiness,while
parasomnla Is an episodic nocturnal behavior that often involves cognitive
disorientation and autonomic and skeletal muscle dlsturbance.
1B
e avnor an syc Iatrrc ISOI' ers
QEUSTIONS
W
1. The percentage of mental illnesses that is encountered at least once in any stage
during childhood is about
A. 10%
B. 20%
C. 30%
D. 40%
E. 50%
3. All the following are true associations between psychiatric illness in childhood and
their treatment EXCEPT
A. attention deficit/hyperactivity (ADHD) and atomoxetine
B. anxiety and antidepressant
C. aggressionand atypical antipsychotic
D. psychosisand typical antipsychotic
E. depression and antidepressant
19
C. resultsfrom neglect
canresolve
D. inInfancy spontaneously .
treatment
E. behavioral ofchance
Isthetreatment
6.inpicadisorder,theMOSTappropriate is
statement
clayandearth
toeating
A. it isonlyrelated
usuallyin childrenbelow2 years
withothereatingdisorders
notpresent
anxietyin a childEXCEPT
cancause
11.Allthefollowingmedicalconditions
A. antihistamine medications
B. hypoparathyrodism
C. prolonged school absences
D. carbonated beverages
E. lead poisoning
13. A school nurse called you to see a 6-yearold boy with school refusal, he is always
crying after parental leaving, refuses to stay in the class room, the school nurse
informed you that she tried her best during the last 4 weeks; and she met with the
parents frequently.
Of the following, the BEST action is to
refer the child to a pediatrics psychologist
start selective serotonin reuptake inhibitor treatment
14. All the following are characteristic features of separation anxiety disorder EXCEPT
not manifested below 3 years of age
common up to 5% of children
W905?
girls are more affected than boys
child thinking often include fear of harm affecting parents
not reported in children above 8 years of age
15. You are asked to explain breath holding spells concept to a staff nurse during
morning round.
0f the following, the BESTstatement; it is a/an
A. manifestation of iron deficiency anemia
B. type of seizure
21
' '
of frustration or. anger
t icalexpressnons
C.
D.
when
manifest
problem
Sisal/i: thechild older
becomes
E.
resolvebyage
which
sortof ticdisorder
16Aconcerned which
ofan18-month-child
parents abrieftonicmovement
expressed
afterbeingupsetandcried. .
All the followingaretrue advuces EXCEPT
investigatepossibilityof iron deficiencyanemia
avoidoverconcernbehavior . .
17 You are evaluatinga 5-year-old child with breath holding spells, the history given
includes pallor with abnormal limb movement lasted for 5 minutes followed by sleep.
All the following are true responses/advices EXCEPT
A reassurance, behavioral instruction to parents and follow up
B. order MRI brain
C. order an ECG
D. order an EEG
E. neurological consultation
havea preexisting
suicide
90%ofyouthswhocomplete
21.Approximately psychiatric
illness.
Of the following, the MOST commonly encountered illness is
A. major depression
B. schizophrenia spectrum disorders
C. conduct disorder
D. chronic anxiety
E. panic disorder
23. All the following are characteristic features of autistic spectrum disorder (ASD)
EXCEPT
defective social communication
highly restricted fixated interests
24. Diagnosisof autistic spectrum disorder (ASD) depends partly but importantly on
assessmentof language. All the following may raise your concern regarding language
development and may indicate ASD EXCEPT
A. absent babbling by 6 months
B. absent gestures by 12 month
C. absent single words by 16 month
D. absent 2-word purposeful phrases by 24 month
E. loss of language or social skillsat any time
26.TheMOST
consistent MRIbrain
ofstructural
statement ofautistic
finding spectrum
disorders (A50) is
A. diffuse brain atrophy
B. increase brain size
C. focal fibrosis
D. white matter degenerativechanges
E. graymatterdegenerativechanges
of autistic
raisethesuspicion
should
27.Allthefollowing disorders
spectrum (ASD)
EXCEPT
A. slblingwithASD
B. playmate concern
C. parental concern
D. caregiver concern
E. pediatrician concern
29. An 18-month-old male toddler was found to be symptomatic for autistic spectrum
disorders(ASD)by routine screeningtesting.The recommendedevaluation includeall
the following EXCEPT
A. physicalexamination for dysmorphic features
8. hearing tests
C. brainCT
D. wood'slamp
E. chromosomalmicroarray
24
of the following, the MOST important next action is
A. physical examination for dysmorphic features
8. hearingtests
C. lead level
D. Wood's lamp
E. chromosomal microarray
31. You are following a 6-yearold boy with autism, he is under structured psychosocial
behavioral training program, there is frequent complains of aggression and self-
injurious behavior.
of the following, the BEST medication to control his behavior is
A. methylphenidate
B. risperidone
C. escitalopram
D. atomoxetine
E. amantadine
33. A parents came to your clinic complaining that their 5-year-old boy had attacks of
frightening with imagination of a snake crawling over him and he is acting as trying to
remove it. You assessed the child and find no acute physical illness and he is
cooperative and quite intelligent.
Of the following, the MOST appropriate explanation for the child behavior is
A. acute phobic hallucination
B. early sign of schizophrenia
C. delusional infestation
D. night terror
E. bipolar disorder
25
Be. avior and Psychiatric Disor . ers
ANSWERS
M Fl
1.(B).
2.(C).Feelingvery sador withdrawn for more than 2 mo.
3.(D). Atypical antipsychotic.
4.(D). Theseare the common medications used in Pediatric psychiatric disorders which
pediatrician should be familiar with. Floxtine is common antidepressant and it is
another selectiveserotonin reuptake inhibitors
S.(A). Rumination disorder is the repeated regurgitation of food, where the
regurgitated food may be rechewed, reswallowed, or spit out, for aperiod of at least 1
mo following a period of normal function. Rumination disorder has also been in healthy
individuals across the life span in addition to infants and those with intellectual
disability.
6.(D). Pica involves the persistent eating of nonnutritive, non-food substances (e.g.,
paper, soap, plaster, charcoal, clay, wool, ashes, paint, earth) over a period of at least 1
mo. The eating behavior is inappropriate to the developmental level (e.g., the normal
mouthing and tasting of objects in infants and toddlers) and, therefore, a minimum age
of 2 yr is suggested. Children with pica are at increased risk for lead poisoning; iron-
deficiency anemia; mechanical bowel problems, intestinal obstruction, intestinal
perforations, dental injury, and parasitic infections. It can be fatal but usually based on
substances ingested.
7.(C). A tic is a sudden, rapid, recurrent (in the same manner), non-rhythmic motor,
movement or vocalization e.g. eye blinking, neck jerking, shoulder shrugging, extension
of the extremities. Onset of tics is typically between ages 4 and 6 yr, in Tourettes
disorder both multiple motor and one or more vocal tics have been present at some
time during the illness, may be concurrently for > 1year. ln chronic or persistent tic
disorder either one or more motor or vocal tics are present during the course of the
disease while in provisional tic disorders the duration is < 1 yr. Chorea is involuntary,
random, quick, jerking movements, most often of the proximal extremities, that flow
from joint to joint (not in same manner), movements are abrupt, non-repetitive, and
arrhythmic and have variable frequency and intensity.
8.(B). Eye blinkingconsidered as simple motor tic, others are sterotypic movements.
Unliketic disorders,the Stereotypicmovementstypically begin within the first 3 yr of
life. These movements resolve over time. Among individuals with intellectual disability,
the stereotyped behaviors may persist for years. Stereotypic movements are a
common manifestation of a variety of neurogenetic disorders, such as Lesch~Nyhan
syndrome,Rett syndrome,fragile X syndrome, Cornelia de Lange syndrome, and Smith:
Magenis syndrome.
9.(C). Thumb sucking is common in infancy and in as many as 25% of children age 2 yr
and 15% of children age 5 yr. Thumb sucking beyond 5 yr may be associated with
sequelae (paronychia, anterior open bite). Like other rhythmic patterns of behavior,
thumb sucking is self soothing. Basic behavioral management, including encouraging
parents to ignore thumb sucking and instead focus on praising the child for substitute
behaviors, is often effective treatment. Simple reminders and reinforcers, such as
giving the child a sticker (or other rewards) for each block of time that he or she does
not suck the thumb can also be considered. Use of noxious agents (bitter salves) this
approach should rarely be necessary. Going deep in social history seems to be non-
appropriate in a well child. Response A is true but incomplete.
10.(A). Generally anxiety classified to a pathological and non-pathological anxiety. In
pathological form it is usually resulted in loss of function (Le. become disabling,
interfering with social interactions, development, and achievement of goals or quality
of life, and can lead to low self-esteem, social withdrawal, and academic
underachievement) also can be associated with somatic symptoms such as weight loss,
pallor, tachycardia, tremors, muscle cramps, paresthesias, hyperhidrosis, flushing,
hyperreflexia, and abdominal tenderness. in this scenario the child never refuses to go
to school or to ride the car, he had no somatic symptoms, good explanation and
reassurance can end the problem.
11.(B). Systemic disorders, hormonal disturbances, and drugs along with carbonated
beverage can cause anxiety in children with it is symptoms and signs such as
tachycardia e.g. hyperthyroidism, hyperparathyroidism, hypoglycemia, central nervous
system disorders (brain tumors), migraine, asthma, lead poisoning, cardiac
arrhythmias, and, rarely, pulmonary embolism, systemic lupus erythematosus,
anaphylaxis, porphyria or pheochromocytoma. Side effect of anti-asthmatic agents,
steroids, sympathomimetics, SSRIs (initiation), anticholinergic agents, and
antipsychoties. Other like diet pills, antihistamines, drug withdrawal, and cold
medicines can cause anxiety.
12.(D). School refusal or phobia it is a common problem, it occurs in approximately 1-
2% of children, associated with anxiety in 40-50% of cases, depression in 50-60%
ofcases, and oppositional behavior in 50% of cases. Younger anxious children who
refuse to attend school are more likely to have separation anxiety disorder whereas
older anxious children usually refuse to attend school because of social phobia.
13.(A). Management of school refusal typically requires parent management training
and family therapy, with school personnel is always indicated; anxious children often
require special attention from teachers, counselors, or school nurses. Parents who are
coached to calmly sending child to school and to reward the child for each completed
day of school are usually successful; in this scenario this option had been used. in cases
of ongoing school refusal, referral to a child psychiatrist or psychologist is indicated.
SSRltreatment may be helpful. Young children with affective symptoms have a good
prognosis.
14.05).Separationanxiety disorder (SAD)is one of the most common childhood anxiety
disorders witha prevalence of 3.5-5.4%. Separation anxiety behavior is
developmentally normal when it begins about 10 mo of age and tapers off by 18 mo. By
3 yr of age, most children can accept the temporary absence of their mother or primary
caregiver. SAD is more common in prepubertal children, with an average age of onset
of 7.5 yr. Girls are more commonly affected than boys. SAD is characterized by
unrealisticand persistent worries about separation from the home or parents.
Numerous somatic symptoms and complaints of subjective distress. Symptoms vary
depending on the childs age; children younger than 8 yr often have associated school
refusal and excessive fear that harm will come to a parent; children 9-12 yr have
excessive distress when separated from a parent; and those 13-16 yr often have school
refusal and physical complaints.
15.(C). Breath holding spells are common during the first years of life and are age-
typical expressions of frustration or anger. Subtypes of breath holding spells include
cyanotic, pallid, or mixed episodes. Cyanotic are the dominant type and may include a
brief loss of consciousness and a very brief tonic-clonic seizure. Pallid spells may be
similar to vasovagal related syncopal events in older children and initiated from similar
stimuli. Iron deficiency with or without anemia may be present and some children with
breath-holding spell respond to iron therapy.
16.(E). All of the above in a sequence of BDCA represent an approach to deal with
classical breath holding spells.
17.(A). in non-classical type of breath holding spells; medical conditions to consider
should include seizures, Chiari crisis, dysautonomia, cardiac arrhythmias (as prolonged
QT interval), and central nervous system lesions. So all the above optional
investigations are justifiable.
18.(C). Lying is a normal behavior between 2-4 years and represents all the above
except the option C.
19.(A). Truancy (running from school) is a never developmentally appropriate. Truancy
may represent disorganization within the home, care taking needs of younger siblings,
developing conduct problems, or emotional problems including depression or anxiety.
Whereas younger children may threaten to run away out of frustration or a desire to
get back at parents, older children who run away are almost always expressing serious
underlying problem within themselves or their family, including violence, abuse, and
neglect. Adolescent runaways are at high risk for substance abuse, unsafe sexual
activity, and other risk-taking behaviors.
20.(A). Hypomanic or manic episodes are not features of major depressive episode. A
period of at least 2 wks is required while in persistent depressive disorder require a
period of 2 years for diagnosis.
21.(A). All mentioned distracters have a role in suicide but major depression is the most
common one.
22.(A). Heart rate <50 beats/min and blood pressure <80/50 mmHg with hypokalemii
and hypophosphatemiaare indications for admission in anorexia nervosa; othel
28
indications may include hypoglycemia, dehydration, temperature <36.1C, <80%
healthy body weight, hepatic, cardiac, or renal compromise, suicidal intent and plan.
23.(E). Autismis typically recognized during the 2nd yr of life but can been seen earlier
than 12 mo if developmental delays are severe. Initial symptoms most frequently
involve delayed language accompanied by lack of social interest or odd play patterns.
Duringthe 2""yr, odd and repetitive behaviorsand the absenceof typical play become
more apparent. It is typical for parents to report that there was no period of normal
development or that there was a history of unusual behaviors. Less commonly (in 20
0.0% of cases), a period of apparently normal development is reported before a loss of
skills appears.
24.(A). Absence of babbling by 12 months is a red flag for language development and
should raise a suspicion of A50 (autistic spectrum disorder).
25.(E). All these mentioned are recognized associations with ASD, it has 3 grads of
severity, early diagnosis usually improve outcome, but those in the severe form
continue to adulthood with problems of independent living, employment, social
relationships, and mental health. Small group (mild form with communication abilities)
can grow up to live selfsufficient lives in the community with employment. Truancy is
separate behavioral disorder.
26.(B). Increase in brain size and increased head circumference had been documented
in autistic children. Other important finding in structural MRI is diffusion tensor
imaging studies suggest aberrations in white matter tract development.
27.(B). Playmate concern is not considered.
28.(C).
29.(C). The recommended evaluation should rule out dysmorphic syndromes;
macrocephaly; hearing problems; tuberous sclerosis and chromosomal abnormalities
and genetic causes like fragile X syndrome and Rett syndrome.
30.(C). Lead level should be order in situation of that, the child is pica eater or he is
living in area with high lead level in soil, air or water. Depending on clinical
presentation, metabolic screen, thyroid stimulating hormone, EEG and specific genetic
studies should be done.
31.(B). The drug of choice is risperidone, while methylphenidate is the drug of choice
for ADHD in normal IQ children, atomoxetine is also a new medication for ADHD, and
escitalopram is used for depression.
32.(E).
33.(A). In children with non-psychotic hallucinations, the symptoms of psychosis are
absent; normal mental status examination is the key for it is diagnosis. Non-psychotic
hallucinations commonly occur in the context of severe traumatic stress,
developmental difficulties, social and emotional deprivation. Hallucinations related to
fantasy, culture believes, grief, hypnogogic hallucinations, night terrors, fever, and
acute phobic hallucinations all are considered normal. In this scenario, the case
typically has an acute phobic hallucination which is benign, common and occurs in
previously healthy preschool children. The hallucinations are often visual or tactile, last
10-60
minutes, at anytimebutmostoftenat night.Thechildis
andoccur
thatbugs orsnakes
arecrawling quite
andmight
frightened complain overhimorherwith
attempt them.Thecause
to remove is The
unknown. differential
diagnosis
and
includes
drugoverdoseorpoisoning,
highfever,encephalitis, psychosis.
Thechildsfearis
notalleviated
byreassurance or
bytheparents physician,
andthe childisnot
amenable
to reason. on and
Findings physical mental
status
examinations
are otherwisenormal,
Symptoms can persist for 13 days, slowly abating over 1-2 wk.
earning isor ers
QUESTIONS
HMED IAWFI
1. The definition of intellectual function is the capacity to think in the abstract, reason,
andcomprehend.Thescoreat whichsignificantweaknesses
problemsolve, in adaptive
skills are evident is equal to or below
A. 55
B. 60
C. 65
D. 70
E. 75
wpnw?nor-epinephrine
troponin
epinephrine
5. Inbetweenspecificlearningdisorders theMOST
(SLD), to treatis
refractory
A. reading
8. mathematics
C. working memory
0. social cognition
31
n
E. writing
Inthehistory withspecific
of children
7. All the followingareredflags learning
disorders(SLD)EXCEPT
A concern
parents academic
about performance
B. inmarks
inconsistency from
report grade tograde
C. withhomeworkactivities
struggling
D. positivevisionor hearingtest
E. positivestandardscreeningtest
are established
8. Allthe following of treatmentin a childwith specific
modalities
learningdisorders(SLD)EXCEPT
of skills
A7. remediation
B. demystification
C. bypass strategies
D. dietary interventions
E strengthening of strength
990!»
must be presentfor at least for 6 mo
mustbe presentin 2 or more
settings
must not be secondaryto another
disorder
32
11. You are assessingan eight~year-old male child with attentionodeflcit/hyperactivity
disorder (ADHD).
0f the following, the LEASTusefultest/investigation is
A. thyroid function test
B. lead level
C. EEG
D. Blood film
E. polysomnography
12. Of the following, the MOST common presenting behavior in glris with attention-
deficit/hyperactivity disorder (ADHD) is
A. inattentive
B. hyperactive
C. impulsive
D. disruptive
E. combined
33
w..-
I Disorders
Leafning
l ANSWERS
$HMED TAWFIQ
mgr???human immunodeficiency
tuberculin skin test
treponemal test
serology
2. You are a newly employee Pediatricianto foster care children (children removed
from their originalfamilies and put in suitable institutes for protection).
Those children have many significant problems, but the MOST common one is
A. chronic medical problems
3. abuse and neglect
C. mental health concerns
D. family relationship problems
E. developmental problems
3. Witnessingviolence,community
violence, and media violence all are detrimentalto
childrenas it increasesthe impact of violence.
Allthefollowing
adverseeffectscanbe increasedby increasingthe impact°f Videnoe
EXCEPT
A. poor school performance
8. symptoms
of anxietyanddepression
C. lower self-esteem
D. cyper bullying
E post traumatic stress disorder
bysome
ofhisnewschool
mates
asa
Ofthefollowingthe "weak".
MOSTlikelVdia ' -
A. school 8n05Is
is
phobia
B. anxietydisorder
C. bullying
D.
hypothyroidism
s.Thefollowing
factors
showpsychosocial
A. lossof
ofwaronchildren
impact EXCEPT
family members
3. separationfrom
community
C. lack of education
D. acute stress reaction
E. displacement
7. Regardlng
the physical
abuse,the physical
signsin abusedchildren
(asbitemarks)
may be found in up to
A. 5%
8. 15%
C. 2596
O. 35%
E. 45%
9. hdilldrenwithphysiwl abuse,whentheirmectedtraumaisbumlnjury.
theMOSTsuggestiveoneis
mfmmmmmmdhexwes
I. presenoeofsplashm
C. unclearhordersolbmn
D. mawmmmm
E. remedies
m gauwmwmmhmmmmwmmwm
(In
A. mwmw
B. posterior rib fracture
C. scapularfracture
D. firstrib fracture
E. clavicle fracture
11. A 3-month-old babygirl admitted to pediatric intensive care unit with severe head
injury (evidentby CTscan),the history given by parents was trivial and not informative.
Yoususpectedchild abuse.
Of the following, the MOSThelpful study to support your suspicion is
A. infantogram (one shot for entire body)
8. lateral and AP view X ray of the spine
C. lateral and AP view X ray of skull
D. radionuclide bone scan
E. MRI brain
12. A 3-monthprecious baby of a keen and highly educated family presented with
history of URTl followed by deterioration of level of consciousness and seizure. CT
brain shows intracranial bleeding with no evidence of skull fracture. Fundocscopy was
normal. Mother gives history of difficult labour but with normal development, mild
hypotonia, and macrocephaly which was reassured initially by general Pediatrician.
Of the following, the MOST likely diagnosis is
A. residual birth trauma
B. arteriovenous malformation
C. acute viral encephalitis
D. glutaric aciduria type 1
E. abusive head trauma (AHT)
13. Retinal hemorrhages are an important marker of abusive head trauma (AHT).
Whenever AHT is being considered, a dilated indirect eye examination by a pediatric
ophthalmologist should be performed.
All the following are characteristic of retinal hemorrhage caused by AHT EXCEPT
A. multiple
8. involve more than one layer of the retina
C. central
D. traumatic retinoschisis
E. various sizes
14. A 3-year-old female child with repeated admissions as bleeding tendency in a form
of spontaneous ecchymosis and bleeding through the nose and urine. She had another
daughter who died because of similar undiagnosed illness. The mother is a staff nurse
and she is highly concerned about her child illness. Lab investigations usually show»l
either PTand/or PTTprolongationwith normal platelets, which usually normalized
after empiric vitamin K administration. The father Is a petrolengineerand never seen
accompanying the family.
Of the following, the MOSThelpful investigation is
A. factor VII assay
B. factor II assay
C. drug level
D. von Willebrand factor assay
E. platelets function test
15. In a child less than 3 years, the diagnosis of failure to thrive (FTI') is considered if
weight is below the 3'd percentile
weight drops down more than 2 major percentile lines
91.0097?
weight for height is less than the 1 percentile
BMI lessthan the 25"1percentile
midarm circumference < 15 cm
F909?neurologic
cardiomyopathy
renal symptoms
symptoms
39
to thrive,recurrent
childwithfailure
19.A 2-year-old andPu'monary
wheezing,
infections.
common
theLEAST
0f thefollowing, of hisillnessis
cause
A. asthma
B. aspiration
C. food allergy
D. cystictibrosis
E. immunodeficiency
20. in a child with failure to thrive, the indications for hospitalization include severe
malnutrition or failure of outpatient management. The period after which the child
needs hospitalization, if he has not been responded to outpatient management, is
about
A. 1-2 wk
8. 4wk
C. 2-3 mo
D. 4mo
E. 5-6 mo
40
Children with Special Needs
ANSWERS
» HMED TAWFIQ
1.(E).
2.(D). All children in foster care have family relationship problems, while 40-60% for
choice A, >70% for choice B, 80% for choice C, and around 60% for choice E.
3.(D). All these (except D) represent the impact of violence on children, while cyber
bullying is a newly emerging form of bullying that takes place using electronic
technology.
4.(C). Showing the power of some children on a weaker one can lead serious
psychological and psychiatric effects. That child was a bullying-victim and should be
protected.
S.(E). It represents physical impact.
6.(A). Physical abuse includes beating, shaking, burning, and biting. Sexual abuse has
been defined as the involvement of dependent, developmentally immature children
and adolescents in sexual activities. Neglect refers to omissions in care, resulting in
actual or potential harm which include inadequate healthcare, education, and
supervision, protection from hazards in the environment, and unmet physical needs
(e.g., clothing, food) and emotional support. Psychological abuse includes verbal abuse
and humiliation and acts that scare or terrorize a child. Trafficking in children (selling
them abroad), for purposes of cheap labor and/or sexual exploitation, expose children
to all of the forms of abuse just mentioned. Child neglect constitutes 78% of the
reports.
7.(C). Sentinel injuries may be noted in approximately in 25% of abused infants and
may precede the diagnosis by weeks or even months from the sentinel event.
8.(C). Features suggestive of inflicted bruises include (a) bruising in a pre ambulatory
infant (occurring in just 2% of infants), (b) bruising of padded and less-exposed areas
(buttocks, cheeks, under the chin, genitalia), (c) patterned bruising or burns conforming
to shape of an object or ligatures around the wrists, and (d) multiple bruises, especially
if clearly of different ages. History is the least dependable because it is either hidden or
skewed.
9.(D). Immersion burn injury patterns; napkin area that spares flexor creases,
immersion stocking burn, immersion glove burn, immersion buttocks burn. It usually
shows a clear delineation between the burned and healthy skin, and uniform depth.
The splash marks are usually absent.
10.(E). It is of low-specificity finding.
11.(D). Of the given options, option D is the best because it detect acute skeletal
injuries (as fractures of posterior rIb fracture that indicate shaking injury) before It
became evident on X ray. If there is suspension in the first scan, another scan can be
41
performed after 2 daysfrom the first one. lnfantogram is not preferable as a onean
shot for entire body. Options8 and C are only a part from skeletal survey,whichis
indicatedat time of arrival and after 2 weeks. MRI brain is best taken 5-7 daysafter
acuteinjury,Miils are helpfulin differentiatingextra axial fluid, determiningtimingof
injuries,assessingparenchymal injury,and identifyingvascularanomalies.
12.(D).Patientswith birthtrauma may have asymptomatic subdurai hematomawhich
may resolveby the end of first month of life. Arteriovenous malformation usuallyhas
subarachnoid hemorrhage. Encephalitis is possibility but unusually present with
lntracraniaibleed.AHTis a possibilitybut presence of hypotonia and macrocephalyand
absenceof retinal hemorrhagemake the diagnosis of Giutaric aciduria type 1 is the
mostlikelyone.
13-lCl-it is often extendingto the periphery(outside the posterior pole). D is highly
characteristic.
14.10. The presentationof medical child abuse (MCA) formerly called Munchausen
syndromeby proxy;may vary in nature and severity. Consideration of MCA shouldbe
triggered when the reported symptoms are repeatedly noted by only one parent,
appropriatetestingfailsto confirma diagnosis,and seemingly appropriate treatmentis
ineffective. Drug level for warfarln may solve the problem in this case, the motheris
staff nurseand she is familial with medical issues,the social history given is suggestive
of disturb relationshipbetween parents.
1548).Weightis belowthe 5 percentile,weightfor heightis lessthanthe5
BMIlessthanthe5 percentile,
percentile, alsodefinesFTT.
mm. The historyof metabolicdisorder is usually acute, severe, and potentiallylife
threatening, recurrent vomiting, liver dysfunction, neurologic symptoms,
cardiomyopathyand myopathy, impairment of special senses, renal symptoms,or
distinctdysmorphicfeatures and/or organomegaly all are my be a part of metabolic
illness.
174A). The first 4 causesare true causesbut the commonest is (A). The choice(El
causesFi'l' with diarrheaand fatty stools.
184A).
19.(E).
20.iCl. Inpatient care may include further diagnostic and laboratory evaluation,all
assessmentand implementationof adequate nutrition, and evaluation of the parent/
child feeding interaction.
growthshouldgenerallybe 2-3 times the averageweightgaii
2143). Minimalcatch-up
(or correctedage.
42
Nutrition
QUESTIONS
ASANEINGHALI
1. Regardingnutritional requirement during childhood,all the following are true
EXCEPT
A. nutrition and growth during 3-6 years of life predict adult stature and some
health outcomes
B. major risk period for growth stunting is between 4 and 24 months of age
C. estimated average requirement (EAR) is the average daily nutrient intake level
estimated to meet the requirements for 50% of the population
D. recommended dietary allowance (RDA) is an estimate of the daily average
nutrient intake to meet the nutritional needs of >97% of the individuals in a
population
E. the 3 components of energy expenditure are the basal metabolic rate, thermal
effect of food, and energy for physical activity
boyis
intake(Al)for the total proteinin a nine-month-old
4.Theadequate
A. 9 gm/day
B. 11 gm/day
C. 13 gm/day
D. 15 gm/day
E. 17 gm/day
43
leucine
methionine
meow?
6.Essential
threonine
valine
alanine
acids
fatty isassociated
deficiency features
withallthefollowing EXCEPT
skinrashes
A. desquamating
B. alopecia
C. thrombocytosis
D. impaired immunity
E. growth deficits
7. Allthefollowingstatements inchildren
ironrequirement
concerning aretrueEXCEPT
optimalintakeof ironfor the first 4 months
A. breastmilk provides
8. ironpresentin animalproteinismorebioavailablethan that foundin
vegetables
C. irondeficiencyis the mostcommonmicronutrientdeficiency
D. cows milk is a good source of bioavailable iron
E. iron supplements can interfere with zinc absorption
8. Zinc deficiencyis associatedwith increased risk for all the following EXCEPT
A. stunting
B. impaired immune function
C. increased risk for respiratory diseases
D. increased risk for diarrheal diseases
E. increased risk for skin infections
9'TheAmerican 0fpediatrics
academy in
thevitaminDintakerecommendation
change
2010 to
A. 200 lU/day
B. 400 IU/day
C. 600 lU/day
D. 800 IU/day
E. 1000 lU/day
the followingEXCEPT
A. otitis media
8. urinarytract
infections
C. septicemia
0. childhoodcancer
E. skin infections
11.Anabsolutecontraindicationto breastfeedingis
hepatitis c infection of the mother
alcohol intake
15. Concerns to be considered among vegetarians are all the following EXCEPT
higher bioavailability of iron
lower 3;; levels
mph???
risk of having lower level of fatty acids
lower levels of calcium and vitamin
lower bioavailability of zinc
D;
45
B. utilization
C. preparation
0. stability
E. availability
17.TheTRUE
hemoglobin is
cutoffto defineanemia
120 g/L for children 6-59 mo
115 g/L for children 5-11 yr
18.TheMOSTcommonlyusedindexfor nutritionalstatusis
A. height-forage
B. weight-for-height
C. body mass index
D. mid-upper arm circumference
E. weight-for-age
91.005?
stunting
cell damage
developmental delay
@909"?
gut produceslessgastricacid and gut enzymes
evrdent responses to infection
reduced red cell mass
signofcelldamage
22.Theoutward inmalnutritionis
A. repeated infections
wasting
mph?
edema
hepatomegaly
facial appearance
23.Therecommended
transition
timefromstabilization
to rehabilitation
phaseinthe
treatmentof malnutrition is
A. 1day
B. 3days
C. Sdays
D. 7days
E. 10 days
24. The WHO recommendation for antibiotic cover in the stabilizationphaseof treating
malnourishedboy free of initial complications is to use
oral amoxicillin
parenteral penicillin
25. During treatment of malnutrition, the signal of entry to the rehabilitation phase is
reduced edema
resolution of infection
27.Thefollowing tobegiven
arerecommended
andtraceelements
vitamins the
during
stabilizationphase of malnutrition EXCEPT
A. iron
47
vitamin A
I"???
follc acid
zinc
multivitamins
insevere
treatment
28.Emergency allthefollowing
includes
malnutrition EXCEPT
shock
hypoglycemia
@999? dehydration
severe anemia
infections
32. Thrombocytopenla
in refeedingsyndromeis mainlya resultof
A. hypophosphatemla
B. hypokalemia
C. hypomagnesemia
D. thiamine deficiency
E. hyperglycemia
Across
allracial thefactor
groups, thatconfers
protection 0139
childhood
against
:3.
gestationalweight gain
highbirth weight
WP???maternal smoking
breast feeding
maternal education
FPO.
6 years
9 years
12 years
36. All the following are childhood obesity-associated comorbidities later in life EXCEPT
A. hypertension
8. gallbladder disease
C. tibia vara
D. asthma
E. type 1 diabetes mellitus
37. Genetic disorders associated with obesity can have all the following features
EXCEPT
A. dysmorphic features
cognitive impairment
FPDP
vision abnormalities
hearing abnormalities
tall stature
49
39.Thefollowing features
physical mightbediscovered
during examination
physical of
anobese boy,
10-year-old andeach the
reflect EXCEPT
morbidity
assomated
A. delaysuggests
developmental geneticdisorder
8. daytimesleepiness sleepapnea
suggests
C. and
polyuria nocturia
suggesttype 1 diabetes
D. blountdisease
hippainsuggests .
nonalcoholicfatty liver disease
E. abdominalpainsuggests
W905?lipoprotein level
liver function tests
renal function tests
41.TheonlyFoodandDrugAdministration
(FDAl-approved
medication
for obesity
in
children <16 yr old is
A. orlistat
B. phentermine
C. topiramate
D. amylin
E. lorcaserin
43. Allthefollowingare
helpfl f°"diaSnosis
ofmarginal
A. darkadaptationtest vitamin
Adeficiency
EXCEPT
B. conjunctival
impression
C. relative cytology
doseresponse
D. plasmaretinol
level
E. diet
history
D. bulgingfontanel
E. echymosis
45.Thefollowingarecharactersticfeaturesof acutehypervitaminosis
A EXCEPT
nauseaand vomiting
drowsiness
rnpnsr?
diplopia
cranial nerve paisies
seizures
46. The following conditions are risk factors for carotenemia EXCEPT
A. liver disease
B. food faddist
C. diabetes mellitus
D. hypothyroidism
E malabsorption
50_Thiamine-responsive
megaloblastic anemia syndrome is a rare disorder, it is
characterized
by the following EXCEPT
A. diabetes mellitus
B. peripheral neuritis
51
C. anemia
megaloblastic
D. sensorineuralhearingloss
E. autosomalrecessiveinheritance
Laere
51.Brown-Vialetto-Vansyndrome(BWLS), disorder
a nEUrologic characterized
sensorineural
deterioration,
neurologic
byprogressive hearing and
loss, pontobulbar
palsy
usually
responds of
withhighdoses
totreatment
niacin
biotin
mpow?riboflavin
pyridoxine
thiamin
53. TheMOSTcharacteristic
manifestationof pellagrais
dermatitis
dementia
W905?diarrhea
anorexia
Iassitude
peripheral neuritis
seborrheicdermatitis
52
55_Pyridoxineis not affected in patients receiving
A isoniazid
8. valproate
C. phenytoin
D. penicillamine
E. corticosteroids
59. Clinical features of cerebral folate deficiency include the following EXCEPT
A. lethargy
B. microcephaly
C. atatxia
D. ballismus
E. blindness
61.Allthefollowing ofhereditary
arefeatures EXCEPT
folatemalabsorpmin
anal ulcers
chronic diarrhea
91.0.05?
failure to thrive
megaloblastic
Opportunistic
anemia
infections
53
Is
folatedeficiency
62.Thebestindicatorofchronic
A. serum folate level
B. RBC folate level
C. urinaryfolate level
D. bone marrow aspiration
level
E. CSF5- methyltetrahydrofolate
63.Effective
supplementation of neuraltubedefectis
offolateforprevention
andcontinued
A. startedat least1 mobeforeconception, throughthefirst2-3
moof pregnancy ..
atleast3 mobefore
B. started conception, afterpositive
to bestopped pregnancy
test.
test and continuedthroughthe first 2-3 moof
C. startedafter positivepregnancy
pregnancy.
. '
to bestoppedafterposutlve
D. startedat least1 mobeforeconception, pregnancy
test.
E. started at least 3 mo before conception, and continued through the first 23
mo of pregnancy
64. The dietary reference intake (DRI) of folate for a healthy eight-month-old infant is
80 microgm/day
150 microgm/day
65.
F909?
200 microgm/day
300 microgm/day
400 microgm/day
WPDFPE
neurotoxicity
nephrotoxicity
severe abdominal pain
respiratory difficulty
66. Importantmechanism
for vitamin812 nutritureincludethe following
maintaining
EXCEPT
direct absorption
acidic pH in the ileum
NPOP?
67.Vitamin
enterohepatic circulation
bindingwith intrinsicfactor
intestinal bacterial synthesis
8,; isimportant
forallofthefollowing
EXCEPT
nematopoiesis
mental development
9909?skeletaldevelopment
psychomotordevelopment
central nervous system myelination
arerealriskfactorsforvitamin8n
68.Allthefollowing EXCEP'I
deficiency
A celiac disease
8. ileal resection
C. lmerslund'grasbecltdisease
0. helicobacter pylori infection
E. useof proton pump inhibitors
70. The dietary reference intake of cobalamin for a healthy eight-monthold infant is
A 0.5 microgm/day
B. 1.0 microgm/day
C. LS microgm/day
D. 2.0 microgm/day
E. 2.5 microgm/day
71. Radiographic
features of scurvyare similar to that of
A. copper deficiency
8. magnesium deficiency
C. vitamin A excess
0. vitamin D excess
E. mercury exposure
75.The arerecognized
following EXCEPT
ofcraniotabes
causes
A. osteogenesis
imperfect
B. rickets
C. syphilis
D. Sotos syndrome
E. normal newborn
causesof ricketsEXCEPT
76. Allthe followingare recognized
A. Mccune-Albrightsyndrome
8. epidermal nevus syndrome
C. tuberus sclerosis
D. neurofibromatosis
E. aluminum-containing antacids
82. The following hereditary forms of rickets may help to diagnose an apparently
healthy mother with the same disease
A. X~ linked hypophosphatemic rickets
B. autosomal dominant hypophosphatemic rickets
C. autosomal recessive hypophosphatemic rickets
D. Fanconi syndrome
E. hereditary hypophosphatemic rickets with hypercalciuria
57
E. PTHlevel
E
vitamin inpremature
deficiency ofthe
one
infantS, isTRUE
following
85.
Regarding theplacenta
notcross
A. it does oflife
inthefirstweek
develops
B.hemolysis .
aCldS
Contains
that
formula
C.premature poly fatty
unsaturated may a
carry
effect hemolysis
against
protective 'infants
iron for
premature
supplementation
D.excessive may the
augment
oxidative stress
E. causesthrombocytopenia
conditions/agents
86.Thefollowing in a similar
areincorporated presentation
to
EXCEPT
bleeding
vitaminKdeficiency
A. liverdisease
B. hereditaryfactorII deficiency
C. high dose salicylate
D. heparin
E. rodentpoison
88. The baby that is least vulnerable to develop vitamin K bleeding tendency is
A. a oldneonate
ofahealthy breastfeeding
thatstartedimmediate
mother
3 ftthreltjediy
er irt
8' Old
Child 21days
Spent inintensive
unit
care total
parenteral
receiving
:Eltrie'vearn ion
a anine-month
OldinfantWithprolonged ofdiarrhea
history antibiotics
andmultiple
therapy
S
-
E. a six-hour neonate o fanepileptic
-
mother ,
thatreceived during
phenobarbital
pregnancy
89. 0 e
A. zinc
B.
andhypogonadism
selemum and dental caries
C. ironandhypothyroidism
o. fluorideandcardiomyopathy
anddecreased
E. manganese alertness
90.OneofthefollowingmatchingisTRUEregarding
thetraceelementexcess
andits
consequence
A. copperand congenital hypothyroidism
B. manganese and hyperuricemia
C. ironand abdominalpain
D. zincand choiestaticjaundice
E. fluorideand nail changes
Nutrition
ANSWERS
SANEIN GHALI
61
39.(C).Type 2 diabetesmellitus is influenced in obesity, not type 1.
40.lE). Glucose, insulin, Triglyceride level, LDL and HDL, total cholesterol, and liver
function tests are the initial markers to be done during evaluation of an obese 12year
girl.
41.(A). Orlistat decreases absorption of fat, resulting in modest weight loss.
Complications include flatulence, oily stools, and spotting.
42.(A). In early vitamin A deficiency, the cornea keratinizes, becomes opaque, is
susceptible to infection, and forms dry, scaly layers of cells (xerophthalmia).
43.(D). The plasma retinol level is not an accurate indicator of vitamin A status unless
the deficiency is severe and liver stores are depleted, in which case low plasma retinol
is likely to be evident.
44.(E). Manifestations of vitamin C deficiency.
45.(E). Nausea, vomiting, and drowsiness; lesscommon symptoms include diplopia,
papilledema, cranial nerve palsies, and other symptoms suggesting pseudotumor
cerebri are the hall mark of acute Hypervitaminosis A. Seizures are unlikely.
46.(E). Malabsorption is not associated with any risk of carotenemia.
47.(A). This triad is rarely reported in infants and young children with severe deficiency
secondary to malignancies or feeding of defective formula.
48.(D). Death from thiamine deficiency usually is secondary to cardiac involvement.
The initial signs are cyanosis and dyspnea, but tachycardia, enlargement of the liver,
loss of consciousness, and convulsions can develop rapidly. The heart, especially the
right side is enlarged.
49.(C). It can develop within 2-3 months of deficient intake. Poor mental status is an
early feature. Hoarseness or aphonia caused by paralysis of the laryngeal nerve is a
characteristic sign. Dry beriberi reflects neuronal involvement while wet beriberi
reflects cardiac involvement.
50.(B). This is an autosomal recessive disorder, characterized by Megaloblastic anemia,
diabetes mellitus, and sensorineural hearing loss.
51.(C). Mutations in genes coding for riboflavin transporter proteins have been
identified in children with BWLS.
52.(C). Rapid clinical response to niacin treatment is an important confirmatory test.
53.(A). Dermatitis, the most characteristic manifestation of pellagra, can develop
suddenly or insidiously and may be .initiated by irritants, including intense sunlight.
54.(B). 0-6 months: 0.1 mg/ day; 7-12 months: 0.3 mg/ day; 1-3 yr: 0.5 mg/day; 4-8 yr:
0.6 mg/day; 9-13 yr: 1.0 mg/day 14-18 yr: 1.2 - 1.3 mg/day.
55.(D). Peripheral neuritis is a feature of deficiency in adults but is not usually seen in
children.
56.(B). Other distracters cause pyridoxine deficiency.
57.(D).
58.(C). Methotrexate, not 6-mercaptopurine.
59.(A).It manifestsusuallyat 4-6 months of age. Other features are irritability ("0t
lethargy), developmental delay, pyramidal tract signs, choreoathetosis and seizures.
62
50.(D). Non hematologic manifestations include glossitis, Iistlessness, and growth
retardation not related to anemia.
61.(A). Hereditary folate malabsorption manifests at 1-3 months of age with recurrent
or chronic diarrhea, failure to thrive, oral ulcerations, neurologic deterioration,
megaloblastic anemia, and opportunistic infections.
62.(B). The normal RBCfolate level is 150-600 ng/ mL of packed cells.
63.(A). All women desirous of becoming pregnant should consume 400-800 pg folic
acid daily; the dose is 4 mg/ day in those having delivered a child with neural tube
defect.
64. (A). The DRls for folate are 65 pg for infants 0-6 months of age and 80ug between
612 months of age. For older children, the DRls are 150 pg for ages 1-3 years; 200 pg
for ages 4-8 years; 300 pg for ages 9-13 years; and 400 pg for ages 14-18 years.
65.03).
66.(B). Alkaline pH not acidic.
67.(C).
68.(E). Use of proton pump inhibitors and/or histamine 2 receptor antagonists may
increase the risk of dehciency but is not a real cause of deficiency.
69.(B). lrritability, developmental delay, developmental regression, and involuntary
movements are the common neurologic symptoms in infants and children, whereas
sensory deficits, paresthesias, and peripheral neuritis are seen in adults.
Hyperpigmentation of the knuckles and palms is another common observation with
812 deficiency in children.
70.(A). 0-6 months: 0.4 rig/day; 7-12 months: 0.5 pg/day; 1-3 yr: 0.9 ug/day 4-8 years:
1.2 pg/day 9-13 years: 1.8 ug/day 14-18 years: 2.4 pg/day.
71.(A).
72.(B).
73.(D). The more specific but late radiologic feature of scurvy is a zone of rarefaction
under the white line at the metaphysis . is zone of rarefaction (Triimmerfeld zone), a
linear break in the bone that is proximal and parallel to the white line, represents area
of debris of broken-down bone trabeculae and connective tissue.
74.(B). Larger doses of vitamin C can cause gastrointestinal problems, such as
abdominal pain and osmotic diarrhea.
7S.(D). Sotos syndrome causes large head.
76.(C). Aluminum-containing antacids cause phosphorus deficiency. McCune-Albright
syndrome, epidermal nevus syndrome, and Neurofibromatosis enhance renal losses.
77.(D).
7S.(D). All other distracters are associated with decreased level of inorganic
phosphorus.
79.(C).
8o.(A).
81.(A). Reduced in chronic kidney disease and dietary phosphorus deficiency only while
elevated in all other forms of rickets.
63
82.(A). Undiagnosed disease in the mother is not unusual in X-linked
hypophosphatemia.
83.(A). Direct measurement of other fat-soluble vitamins (A, E, andK) or indirect
assessmentof deficiency (prothrombin time for vitamin K deficiency) is appropriate if
malabsorption is a consideration.
84.(B). The level of 1, 250 is only low when there is severe vitamin D deficiency.
85.(D). lt crossesthe placenta significantlyduring the third trimester, which brings
premature infants at risk. Hemolysis in premature infants typically started in the
second month of life. Formulas containing poly unsaturated fatty acids may lead to
exacerbation of deficiency as they make the red blood cells susceptible to oxidative
stresses. in premature infants, deficiency causes hemolytic anemia and
thrombocytosis, not thrombocytopenia.
86.(D). Anticoagulant warfarin and rodent poisons contain Coumarin derivatives that
inhibit the action of vitamin K by preventing its recycling to an active form.
87.(C). Factor VII has the shortest half life of the coagulation factors.
88.(A). Delayed breast feeding is a risk factor to vitamin K bleeding tendency.
89.(A). Selenium deficiency causes cardiomyopathy and myopathy; Iron deficiency
causes anemia and learning impairment; Manganese deficiency causes
hypercholesterolemia and weight loss; Fluoride deficiency causes dental caries.
90.(C). Copper excess causes acute (Nausea, vomiting and hepatic coma) and chronic
toxicity (liver and brain injury); Manganese excess causes neurological manifestations
and cholestatic jaundice; Zinc excess causes abdominal pain, diarrhea and vomiting;
Fluoride excess causes dental fluorosis.
Fluid and Electrolyte Disorders
QUESHONS
HTAN ALOBAIDY
5.TheMOSTdevastating is
of untreatedhypernatremia
consequence
A. brain hemorrhage
seizures
wpnp
central pontinemyelinolysis
extra pontinemyelinolysis
brain edema
with thefirst
dehydration, isrestoration
priority of
5_Inachild hypernatremic
intravascular volume by
A. 3% saline
8. normal saline
C. Iactated Ringersolution
D. 1/3 glucose saline
E. 1/2 glucose saline
ispresent
7.Pseudohyponatremia EXCEPT
inallthefollowing
A. multiple myeloma
B. immunoglobulininfusion
C. protein losing enteropathy
D. hypertriglyceridemia
E. hypercholesterolemia
8. Hypervolemichyponatremiais causedby
cirrhosis
cerebral salt wasting
WPOP?pseudohypoaldosteronism type |
obstructive
bowel
9. Asymptomatic
uropathy
obstruction
hyponatremia is seen in
A. cirrhosis
B. tap water enema
C. child abuse
D. hyperglycemia
E. tubule -interstitial nephritis
ofinappropriate
Syndrome antidiuretic
hormone
secretion ischaracterized
(SIADH)
:0.
V
extravascular volume expansion
high serum uric acid
[9093
gastroenteritis
diabetic ketoacidosis
loop diuretic
67
E. appearanceof a U wave
include
ofhypermagnesemia
manifestations EXCEPT
allthefollowing
18.Clinical
A. hypotonia
B. hyporeflexia
C. sleepiness
D. hypertension
E. vomiting
include
ofhypermagnesmia
19.Treatment EXCEPT
allthefollowing
A. intravenous hydration
B. loop diuretics
C. Kayexalate
D. exchange transfusion
E. intravenous calcium gluconate
@909?renal insufficiency
cow's milk intake
hypoparathyroidism
mph?»sevelamer
lanthanum
aluminum
27. In metabolic alkalosis secondary to gastric loss, all the following mechanisms
prevent renal bicarbonate loss EXCEPT
A. mineralocorticoid escape phenomenon
8. reduce GFR
C. sodium and bicarbonate resorption
D. increase aldosterone level
E. hypokalemia
serum
Measuring ofrenin
concentrations children
differentiates
andaldosterone
29'-
Wlthmetabolic areelevatedin
bothreninandaldosterone
alkalosis;
69
renovascular disease .
aldosteronism
glucocorticoid-remediable
30.Increased ofC02
production EXCEPT
inallthefollowing
occurs
fever
emesis
of respiratoryalkalosisinclude
31.CNSmanifestations
A. psychosis
B. anxiety
C. asterixis
D. paresthesia
E. hallucinations
70
intemperature
increase above
38Cleads
toincrease
inmaintenance
:yc
1:221:23;
A. 5-10%
B. 10-15%
C. 15-20%
D. 20-25%
E. 25-30%
normal saline
ringer lactate
37. The MOST common manifestation of cerebral edema from an overly rapid decrease
of serumsodium concentration during correction of hypernatremic dehydration is
A. irritability
B. hyperreflexia
C. spastisity
D. seizure
E. coma
71
- FluidandElectrolyte
Disorders
ANSWERS
QAHTAN ALOBAIDY
1.(A).
2.(B). There is an increase in venous hydrostatic pressure from expansion of the
intravascular volume, which is caused by impaired pumping by the heart, and the
increase in venous pressure causes fluid to move from the intravascular space to the
interstitial space.
3.(E). Even though the amount of sodium resorbed in this segment is less than in any
other segment.
4.(o).
5.(A). As the extracellular osmolality increases, water moves out of brain cells, leading
to a decrease in brain volume, this decrease can result in tearing of intra cerebral veins
and bridging blood vessels as the brain moves away from the skull and the meninges,
patients may have subarachnoid, subdural, and parenchymal hemorrhages. Seizures
and brain edema are more common during correction of hypernatremia.
6.(B). Repeated boluses of normal saline (10-20 mL/kg) may be required to treat
hypotension, tachycardia, and signs of poor perfusion (poor peripheral pulses and
capillary rehll time).
7.(C). Hypoalbuminemia caused by gastrointestinal disease (protein losing enteropathy)
cause hypervolemichyponatremia, while pseudohyponatremia is a laboratory artifact
that is present when the plasma contains very high concentrations of protein (multiple
myeloma, intravenous immunoglobulin infusion) or lipid.
8.(A). Other distracters are causes of hypovolemic hyponatremia.
9.(D). Because the manifestations of hyponatremia are a result of the low plasma
osmolality, patients with hyponatremia resulting from hyperosmolality do not have
symptoms of hyponatremia.
10.(D). Because SlADH is a state of intravascular volume expansion, low serum uric acid
and BUN levels are supportive of the diagnosis, the kidney increases sodium excretion
in an effort to decrease intravascular volume to normal; thus, the patient has a mild
decrease in body sodium(urine sodium >30 mEq/L).
11.(C). This phenomenon is exaggerated with thrombocytosis because of potassium
release from platelets, for every 100,000/m3 increase in the platelet count; the serum
potassium level rises by approximately 0.15 mEq/ L.
12.(C).
13.(8). Spurious hypokalemia occurs in patients with leukemia and very elevated white
blood cell counts if sample for analysis is left at room temperature, permitting the
white blood cells to take up potassium from the plasma.
72
14.(B).The gastric loss of hydrochloric acid (HCI), leading to a metabolic alkalosis and a
state of volume depletion. The kidney compensates for the metabolic alkalosis by
excreting bicarbonate in the urine, but there is obligate loss of potassium and sodium
with the bicarbonate. The volume depletion raises aldosterone levels, further
increasing urinary potassium losses and preventing correction of the metabolic
alkalosis and hypokalemia until the volume depletion is corrected.
15.(C). Hypokalemia stimulates renal ammonia production, an effect that is clinically
significant if hepatic failure is present, because the liver cannot metabolize the
ammonia. Hypokalemia impairs bladder function, potentially leading to urinary
retention.
73
deficiency, and 1Iii-hydroxysteroia
hydroxylasedeficiency,11p-hydroxvlase
dehydrogenase deficiency.
30(8).
maybe partiallyrelatedto the reduction
tetany,andseizures
31.(D).Theparesthesla,
in ionizedcalciumthat occursbecausealkalemia causesmore calciumto bind to
albumin.
32.(C). Hypophosphatemlacauserespiratory muscle weakness.
33.(A).
34(5).
3548).
36.(A). Ringerlactateshouldnot be used becausethe lactate would worsenthe
alkalosis.
31(0).
74
Pediatric wrug erapy
QUESTIONS
ALI n ALAARJ
2. Postoperative nausea and vomiting (PONV) may be related to the stress, trauma of
surgery, and pain.
Of the following, the MOST likely drug that cause PONV is ,
A. ketorolac
droperidol
none
acetaminophen
opioid analgesics
local anesthetic agents
4.Malignant isanacute
hyperthermia thatistriggered
syndrome
hypermetabolic by
inhalational
anestheticagentsand succinylcholine. _ .
0fthefollowing, to
clue the of
risk malignant '5
hypertherma
the MOST
recognized
A. hypercarbia
8. rapidonsetof fever
C. ventricularfibrillation
D. acutehemoglobinurla
E. rigidclenching
of the massetermuscles
76
common
ismost
apnea within thelst12hrafter and
surgery
has
SPostanesthetlc
Infants
upto 48 hr later.
Inpremature
reorted .
been for
emergency
(except
avoided
be
should of
Generaypanesthesia m
surgery)
fUII-term
infants than
younger age
postconceptual
A. 44wk
8. 46 wk
C. 48 wk
0. 50wk
E. 52 wk
6.Upper illness
respiratory canincrease
(URI) forupto6 wkinboth
reactivity
airway
normal andchildren
children ofreactive
withahistory disease.
airway
betaken
should
Allthefollowing a childforOperation
whenpreparing
inconcern with
historyof URIEXCEPT
fever
Injectedsclerae
8. Thechildwithasthma
should
be freeof for at leastseveraldaysbefore
wheezing
even
Ifthis
necessitates
anincrease
inB-agonist of
andtheaddition
dosage
:rfsegy,
arm 5.
appropriate
IIIIOST steroids
preoperative regimeinchildrenWith
Ithe
it;:af(°£::?r8e' asthma
ecelvmg steroidwithintheSt
orwhohavereceived
therapy
year)Is
1mg/kg 24and
given 12hrbefore
1;.pregnisone,
2mg/kg surgery
24and12hr
given before
surgery
c, ggiamsose,
0.6mg/kg 24and12hrbefore
0: hydroczmasson, given surgery
oneIV,5 mg/kggiven24and12
6. hydrocortisone hrbeforesurgery
IV,10mg/kg given24and12hrbeforesurgery
78
9. Childrenwithanteriormediastinal
masses
are at seriousriskfor airwaycompromise,
cardiactamponade,and vascularobstruction. The indicationto prohibitgeneral
isCTscanshowingcompression
anesthesia of the airwayat the carina
230%
240%
WPDP?250%
260%
270%
11. Of the following, the MOST acceptable hematocrit value for routine elective
anesthesia is
A. 228%
B. 230%
C. 232%
D. 234%
E. 236%
13'Aspiration
ofgastric
contents disaster
isa perioperative on
and,if superimposed
lung mayberapidlyfatal.
disease, _
Allthefollowing fastingEXCEPT
for preoperative
aretrueaboutthe guidelines
A. clear,sweet liquids--- 2hr
3. breastmilk ----------- 4hr
C. infantformula ------- 6hr
0. solidfood --------- 8hr
5- gelatin............... 10hr
77
ofpoisoning
isasign
14.Nystagmus EXCEPT
withallthefollowing
cocain
alcohols
£119.05?
ketamine
anticonvulsant
dextromethorphan
Poison
to the National
15.According of the American
DataSystems Association
of
Poison
Control
Centers, 50%of occurs
approximately poisoning in children
52 yr old
54 yr old
.MPDP? 56 yr old
58 yr old
510 yr old
18.
POP? salicylates
paraldehyde
isopropyl alcohol
91.0059ng
salicylate
imipramin
acetaminophine
organophosphates
78
C. hypnotics
D. organophosphates
E. tricyclic antidepressant
24.Allthefollowing shadow
mayhavea radiopalilue EXCEPT
°" KUB
poisonings
A. lead
8. arsenic
C. salicylates
D. calcium carbonate
E. enteric-coated pills
25.Prolonged With
PRintervalisa signof poisoning
A. lithium
3. fluconazole
79
C. erythromycin
D. carbamazepine
E. diphenhydramine
QTc
26.Prolongedisasign with
ofpoisoning
amiodarone
carbamazepine
F1905?
diphenhydramine
cardiacglycosides
tricyclicantidepressants
butearlyandappropriate
fewtoxins,
forrelatively
areavailable
27.Antidotes useofan
thepoisoned
inmanaging
isa keyelement
antidote patient.
Thefollowingmatching aretrue EXCEPT
A. B Blockers -------- glucagon
B. opioids----------- naloxone
C. iron ..._....._...-..- deferoxamine
D. lead------ BAL(dimercaprol)
E. isoniazid -------- thiamine
and antidotesEXCEPT
aretrue regardingpoisoning
28. Thefollowingmatching
A. acetaminophen--- N-Acetylcysteine
B. anticholinergics ----- physostigmine
C. benzodiazepines ---- nalorphene
0. carbon monoxide-mun 100% oxygen
E. organophosphates ---- pralidoxime
29. The majority of poisonings in children are from ingestion, although exposures can
also occur via inhalational, dermal, and ocular route.
All the following are true about decontamination EXCEPT
A. the goal is to minimize absorption of the toxic substance
B. decontaminationshouldbe routinelyemployed for every poisoned patient
C. water shouldnot be usedfor decontamination after exposure to phosphorus
theefficacy
oftheintervention
decreases timesince
withincreasing twill-"5re
ED.
theSPECifiC
method, on
depends the of the
properties toxinandtherouteof
exposure
30.Allthefollowing maycauseQTcprolongation
poisonings EXCEPT
A lithium
B. cisapride
C. amiodarone
D. antipsychotics
E. clarithromycin
33. The majority of poisonings in children are from ingestion, although exposures can
also occur via inhalational, dermal, and ocular routes.
All the following are described methods of GI decontamination EXCEPT
gastric lavage
whole-bowel irrigation
34. After a review of the evidenceand assessmentof the risksand benefits of ipecac
use, the American Academy of Clinical Toxicology have published statements that
advise to
use ipecac with caution
use ipecac in all poisoning
35. Although gastric lavage was used routinely for many years, in most clinical scenarios
of poisoning, the use of gastric lavage is no longer recommended due to all the
following EXCEPT
81
painful
time-consuming
36.Allthefollowing maycause
poisonings
charcoal
ofactivated
EXCEPT
QRSprolongation
sotalol
quinidine
wpop? chloroquine
propranoloi
procainamide
@905? alcohols
hydrocarbons
benzodiazepines
40. Allthefollowing
drugstoxicitycanberemovedby hemodialysis
EXCEPT
A. iron
B. methanol
C. salicylates
D. theophylline
E. valproicacid
82
41. lntralipid emulsions therapy (IET) act by squesteringfat-soluble drugs and
decreasingtheir impact at target organs thus enhance the elimination in case of drug
toxicity.
All the following can be eliminated by IET EXCEPT
A diltiazem
B. verapamil
C. amiodrone
D. imipramine
E. amitriptyline
42. All the following methods can be used to enhance the elimination of toxins EXCEPT
hemodialysis
urinary alkalinization
44. In toxic ingestion of acetaminophen, the serum level should be measured after
2 hr
4 hr
F909? 6 hr
8hr
10 hr
FPOF
anticonvulsant
carbon monoxide
dextromethorphan
83
elevated alanine aminotransferase
WP?!
high international normalized ratio
right upper quadrant abdominal pain
elevated aspartate aminotransferase
52. Salicylate ingestions are classified as acute or chronic, and acute toxicity is far more
common in pediatric patients.
Which of the following is not a recognized feature of salicylate poisoning?
tinnitus
tachypnea
W999? diaphoresis
bradycardia
hyperthermia
53. A 2.5 yearold- child brought to emergency room immediately upon finding the boy
with open bottle of some drugs (used by his grandfather). The child has vomited once,
fussy, and lethargic. Physical evaluation reveals a 13kg child who has tachypnea and
tachycardia. Laboratory results include a blood sugar=225mgldl, pH=7.S, PC02=20
mEq/L, and HC03=20 mEq/L.
0f the following, the MOST likely offending drug is
A. iron
8. atropine
C. salicylate
D. methylxanthine
E. tricyclic antideprssnt
wpnw? antidepressants
methylxanthines
calcium channel blockers
55. In salicylate poisoning, hyperglycemia (early) and hypoglycemia (late) have been
described.
Of the following, the MOST appropriate clue to administer IV glucose is
A. vomiting
B. convulsion
C. paipitation
D. diaphoresis
E. altered mental status
havean insidious
56.Chronicsalicylism andpatientscanshowmarked
presentation,
levelsthanin acutetoxrcrty.
lowersalicylate
toxicityat significantly
Ofthe following,the MOSTappropriate primarymodeof therapyIn chronicsalicylism
iS
IV glucose
hemodialysis
gastric decontamination
57. ibuprofen is well tolerated even in overdose. The acute dose of ibuprofen that
result in more serious effects (altered mental status and metabolic acidosis) is
A. >100 mg/kg
B. >200 mg/kg
C. >300 mg/kg
0. >400 mg/kg
E. >500 mg/kg
58. In acute toxic ingestion of ibuprofen, symptoms usually develop within 4-6 hr of
ingestion and resolve within 24 hr.
All the following can result from acute ingestion of variable dose of ibuprofen EXCEPT
A. gastric ulcers
B. CNS depression
C. abdominal pain
0. renal insufficiency
E. nausea and vomiting
99???
directly proportional to its viscosityand volatility
inversely proportional to its viscosity,and directly proportionalto its volatility
directly proportional to its viscosity,and lnverselyproportionalto Its volatility
62. Hydrocarbons with low viscosity and high volatility (kerosene, gasoline) spread
rapidly across surfaces and cover large areas of the lungs when aspirated.
ofaspirated thatcanproduce
chemicals
hydrocarbon is
Injury
significant
IThe
esst qruantity
an
63. Certain hydrocarbonshave unique toxicities and can cause symptoms after
ingestion, inhalation, or dermal exposures.
leukemiais long-term
Of the following,the MOST likelycauseof acute myelogenous
exposure to
gasoline
benzene
POP? kerosene
nitrobenzene
methyl alcohol
mayresultfromexposureto
64. Methemoglobinemia
A. gasoline
B. benzene
C. kerosene
D. nitrobenzene
E. carbon monoxide
ofhydrocarbons
manifestations
areclinical
65.Allthefollowing EXCEPT
poisoning
A. transientmildCNSdepression
isthefirstclinical
8. cough finding
C. chest
radiographs normal
maybeinitially
2-3wkafter
canappear
D. pneumatoceles exposure
87
and
E.fever leukocytosis superinfection
bacterial
indicate
mayresult
66.Dysrhythmias to
fromexposure
A. benzene
B. kerosene
C. nitrobenzene
D. carbon tetrachloride
E. halogenatedhydrocarbons
monoxide
67.Carbon (CO) odorless
isacolorless, duringthecombustion
gasproduced
the greaterthe
fuel (thelessefficientthe combustion,
of anycarbon-containing
amount of C0 produced).
All the followingaretrue aboutCOpoisoningEXCEPT
A. COdisplacesnitric oxide (NO) from proteins
NO is responsiblefor headache,syncope, and hypotension
HbCO levels are Well correlated with clinical signs of toxicity
68. Early symptoms of carbon monoxide (CO) poisoning are nonspecific and include
headache, malaise, nausea,vomiting, and the skin may appear
A. blue
8. pale
C. normal
0. grayish
E. cherry-red
none?salivation
bradycardia
urine retention
73. Nicotinic signs and symptoms of organophosphates poisoning include all the
following EXCEPT
A. tachycardia
B. hypotension
C. fasciculation
D. hypoventilation
E. muscle weakness
of digoxintoxicityEXCEPT
75.Allthe followingare donein the initialassessment
A. ECG
B serumcalcium
C. serum potassium
D. renal function test
E. serum digoxinlevel
EXCEPT
maycausehypoglycemia
76.Allthefollowingpoisoning
A. quinine
89
ethanol
999!
sulfonylureas
beta Blockers
calciumchannelblockers hyperglycemia
77.Allthefollowing aretrueEXCEPT
matching
P salicylates -- sodium bicarbonate
8. organophosphates atropine
C. methemoglobinemia methylene blue
D. calciumchannel ------~
blockers insulin
E antidepressants
tricyclic flumazenilNaHco3
index,therapeuticplasmadigoxin
78. Digoxinhas a very narrowtherapeutic
toxicand a level>6
are0.5-2.0ng/mL;a level>2 ng/mLis considered
concentrations
ng/mLis consideredpotentiallyfatal.
increaseserumdigoxinconcentrationEXCEPT
Allthe followingmedications
P tetracyclin
B. amiodarone
C. itraconazole
D. clarithromycin
E. spironolactone
In toxicity,Fabfragments
bindfreedigoxinin boththe intravascular
andthe
:79. digoxin
Interstitial space to form a pharmacologicallyinactive complex that is subsequently
renally eliminated.
All the followingare indicationsof Fabfragments EXCEPT
renal failure
hypertension
meow?ingestion >4 mg
K value>S-5.SmEq/L
life threateningdysrhythmias
82. A 2.5 year-old-boy presented with tachycardia, pallor, and fatigue. The history
reveals excessive iron ingestion with unknown duration.
Of the following, the MOST likely time of ingestion is
A. 30 min-6hr ago
8. 6hr-24 hr ago
C. 12hr-36 hr ago
D. 36hr-48hr ago
E. 48hr-72hr ago
@905? vancomycin
carbon monoxide
elemental mercury
91
Rwave
E. prominent aVR
inlead
common
MOST
86The ofdeath
cause overdose
(TCA)
antidepressant
intricyclic is
seizures
renal injury
hype?respiratoryfailure
refractoryhypotension
ventriculararrhythmias
with
child
87.Asymptomatic tricyclic toxicity,
antidepressant beobserved
should with
cardiac
continuous andserial
monitoring least
forat
EC65
4hr
6hr
wgnw?8hr
10hr
12hr
areoralsupplement
88. Probiotics number
or a foodproductthat containsa sufficient
of viablemicroorganisms that are
(live microorganisms most often referred to as
"friendly" or "good" bacteria).
Probioticsare indicated in all the following EXCEPT
A. constipation
B. irritable bowel syndrome
C. protien losing enteropathy
D. antibiotic-associated diarrhea
E. inflammatory bowel disorders
89. Dietarysupplements
(asherbs)are commonlyusedand may be helpfuladjuncte
treatments for common childhood problems.
All the following matching are true EXCEPT
A. Aloe vera lice
8. Ginger -------- -------- Nausea
C. Chamomile---------- dyspepsia
D. Tea tree oil --------- acne remedies
E. Peppermint-- Irritablebowelsyndrome
r' 3 Pediatric
DrugTherapy
ANSWERS
KHALID ALAARJI
93
25.(A). Erythromycin and fluconazole may cause QTc prolongation while
diphenhydramine and carbamazepine may cause QRS prolongation.
26.(A). The other distracters may cause wide QRS.
27.(E). Pyridoxine is antidote for isoniazid.
28.(c). Flumazenil is antidote for benzodiazepines.
29.(B). Decontamination should not be routinely employed for every poisoned patient.
Instead, careful decisions regarding the utility of decontamination should be made far
each patient and should include consideration of the toxicity and pharmacologic
properties of the exposure, the route of the exposure, the time since the exposure, and
the risks vs the benefits of the decontamination method.
30.(A). Lithium may cause PR interval prolongation.
31.(C). Dermal decontamination, especially after exposure to adherent or lipophilic
(e.g., organophosphate) agents, should include thorough cleansing with soap and
water. Water should not be used for decontamination after exposure to highly reactive
agents, such as elemental sodium, phosphorus, calcium oxide, and titanium
tetrachloride.
32.(A). Gastrointestinal decontamination strategies are most likely to be effective in
the lst hour after an acute ingestion. GI absorption may be delayed after ingestion of
agents that slow GI motility (anticholinergic medications, opioids), massive pill
ingestions, sustained-release preparations, and ingestions of agents that can form
pharmacologic bezoars (e.g., enteric-coated salicylates).
33.(E). Whereas singledose activated charcoal is used as a method of
decontamination, multiple doses of activated charcoal (MDACs) can help to enhance
the elimination of some toxins.
34.(C).
3S.(C). Gastric lavage can induce bradycardia via a vagal response to tube placement.
36.(A). Sotalol may cause QTc prolongation.
37.(E). Charcoal is activated via heating to extreme temperatures, creating an
extensive network of pores that provides a very large adsorptive surface area. Many,
but not all, toxins are adsorbed onto its surface, thus preventing absorption from the GI
tract.
38.(B).
39.(C). Urinary alkalinization (UA) enhances the elimination of some drugs that are
weak acids by forming charged particles that are trapped within the renal tubules
and thus excreted. Urinary alkalinization is accomplished via a continuous infusion of
sodium bicarbonatecontaining intravenous fluids, with a goal urine pH of 7.5-8.
Alkalinization of the urine is most useful in managing salicylate and methotrexate
toxicity.
AO.(A).Toxins that are amenable to dialysis have the following properties: low volume
of distribution (<1 L/kg), low molecular weight, low degree of protein binding, and high
degree of water solubility.
41.(C). Lipophilic drugs are potentially bound by lntralipid emulsions, including calcium
channel blockers (verapamil and diltiazem) and tricyclic antidepressants.
42.(C). Enhancing elimination is a potentially lifesaving intervention that results in
improved clearance of a poison that has already been absorbed. Whole bowel irrigation
used for gastrointestinal decontamination.
43.(C). In acetaminophen overdose, glutathione stores are overwhelmed, and free N~
acetyl-p-benzoquinone imine is able to combine with hepatic macromolecules to
produce hepatocellular necrosis.
44.(B). Acetaminophen levels obtained <4 hr after ingestion, unless nondetectable,
are difficult to interpret and cannot be used to estimate the potential for toxicity.
45(8). CNS toxicity of antidepressant can include lethargy, coma, myoclonic jerks, and
seizures.
95
$8.(A).GI bleedingandulcershavebeen describedwith chronicuse;they are rarein
the settingof acute ingestion.
89(5). The most important manifestationof hydrocarbon toxicity is aspiration
pneumonitisvia inactivationof the type II pneumocytes and resulting surfactant
deficiency.
60.(A).Mercury,lead,arsenic,and bismuthcausegum lines.
andhighvolatility,suchas mineralspirits,naphtha,kerosene,
61.(D).Withlowviscosity
andlampoil,spreadrapidlyacrosssurfacesand cover large areasof the lungs
gasoline,
when aspirated.
62.(A).Onlysmallquantities(<1 mL) of suchchemicalsneed be aspirated to produce
significantinjury.
63.(B). Benzeneis known to cause cancer, most commonly acute myelogenous
leukemia,after long-termexposure.
64.(D). Nitrobenzene, aniline, and related compounds can produce
methemoglobinemia.
65.(E). Fever and leukocytosisare common accompanying signs in patients with
pneumonitis anddon't necessarily implybacterialsuperinfection.
66.(E). Halogenatedhydrocarbons(which contain a chlorine, bromine, or fluorine), can
sensitizethe myocardiumto the effects of endogenous catecholamines. This can result
in dysrhythmias and sudden sniffingdeath".
67.(C).C0 displacesoxygenand createsa conformational change in hemoglobinthat
impairsthe deliveryof oxygento the tissues,leadingto tissue hypoxia. HbCO levelsare
not well correlated with clinical signs of toxicity, likely because CO interacts with
multipleproteinsin additionto hemoglobin.
68.(E). At higher exposure levels, patients can develop mental status changes,
confusion, ataxia,syncope,tachycardia,and tachypnea. Severe poisoning is manifested
by coma, seizures,myocardial ischemia, acidosis, cardiovascular collapse, and
potentiallydeath.Onexam,patientsmight have cherry-red skin.
69.(8). in ambient air, the average half-life of HbCO is 4~6 hr. This is dramatically
reducedto 60-90 min by providing100% oxygen at normal atmospheric pressuresvia a
non-rebreatherfacemask.Severelypoisoned patients might benefit from hyperbaric
oxygen(HBO),whichdecreasesthe half-life of HbCOto 20-30 minutes.
maycauseQRSprolongation.
70.(B).Propranoloi
71.(A).Theseverityof the corrosiveinjurydependson the pH and concentrationof the
aswellasthelengthof contacttimewith the product.Agentswith a pHof <2
product
or >12are mostlikelyto producesignificantinjury.
72.(E). A commonlyused mnemonicfor the symptoms of cholinergicexcessat
muscarinicreceptorsis DUMBBEIS,which stands for diarrhea/defecation, urination,
bradycardia,emesis,lacrimation,and salivation.
miosis,bronchorrhea/bronchospasm,
73.(B).Hypertension.
74.(C).Atropinedosingisprimarilytargetedto dryingthe respiratorysecretions.
75.(B). The serum digoxin level should be assessed at least 6 hr after ingestion and
carefully interpreted in the setting of clinical symptoms because the digoxin level alone
does not entirely reflect the severity of intoxication. In acute ingestions, serum
potassium is an independent marker of morbidity and mortality, with levels >5.5 mEq/L
predicting poor outcomes. In chronic toxicity, serum potassium is less useful as a
prognostic marker and may be altered due to concomitant use of diuretics.
76.(E). Calcium channel blockers may cause hyperglycemia.
77.(E). Sodium bicarbonate is antidote for tricyclic antidepressants.
78.(A). Numerous drug interactions affect plasma digoxin concentrations. Medications
known to increase serum digoxin concentrations include the macrolides, erythromycin
and clarithromycin, spironolactone, verapamil, amiodarone, and itraconazole.
79.(B). Clinically significant hypotension or other cardiovascular instability.
80.(E). The severity of an exposure is related to the amount of elemental iron ingested.
Ferrous sulfate contains 20% elemental iron, ferrous gluconate 12%, and ferrous
fumarate 33%. Multivitamin preparations and children's vitamins rarely contain enough
elemental iron to cause significant toxicity.
81.(D). Pediatric patients who ingest >40 mg/kg of elemental iron should be referred to
medical care for evaluation, although moderate to severe toxicity is typically seen with
ingestions of >60 mg/kg.
82.(B). The 2nd stage, 6-24 hr after ingestion, is often referred to as the quiescent
phase, as GI symptoms typically have resolved. However, careful clinical exam can
reveal subtle signs of hypoperfusion, including tachycardia, pallor, and fatigue.
83.(B). The other distracters may cause erythema.
84.(D).
85.(B). Sinus tachycardia is the most common cardiovascular manifestation of toxicity;
however, patients can develop widening of the QRS complex, premature ventricular
contractions, and ventricular arrhythmias. Refractory hypotension is a poor prognostic
indicator and is the most common cause of death in TCA overdose.
86.(D). Refractory hypotension is a poor prognostic indicator and is the most common
cause of death in TCA overdose.
87.(B). Children who remain completely asymptomatic with normal serial ECGsmay be
candidates for discharge after 6 hr of close observation.
88.(C). Probiotlcs also indicated in Clostridium difficileassociated diarrhea.
89.(A). Tea tree oil ~ Antibacterial (acne remedies), pediculicide (lice), and Aloe vera -
~- Mild burns.
97
cute I
QUESTIONS
WW
a
for
reason
common child
sick is
visit
MOST
1.The
A. fever
8. alteredmentalstatus
C. vomiting
distress
D. respiratory
pain
E. abdominal
medical the
emergencies can
rate
survival good
with
increase
2.Inpediatrics effective,
ifrapid, IS
done.
(CPR)
resuscrtatlon
cardiopulmonary
outcome
neurological
ratecanreachupto
Thesurvival
A. 10%
B. 30%
C. 50%
D. 70%
E. 90%
tounwitnessed
3.Thefirstresponse infantisto
unresponsive
A. activateemergencyresponsesystem
8. check pulse
C. start rescue breathing
D. start chest compression
E. do endotracheal intubation
5.After
activation
ofemergenw
medical Child
tounresponsive
action
thenext
system,
is to
A. assesthe air ways
B. check pulse
C. give rescue breathings
D. start chest compression
E. attach automated external defibrillator (AED) to find shakable rhythm
7. The correct statement regarding the lower limit of systolic blood pressure for a
neonate is
240 mm Hg + (2 x age)
250 mm Hg 4»(2 x age)
."IPOP?
260 mm Hg + (2 x age)
270 mm Hg + (2 x age)
280 mm Hg + (2 x age)
8. The MOST common precipitating event for cardiac instability in infants and children
IS
electrolyte disturbances
trauma
F1905?
respiratory
poisoning
myocarditis
insufficiency
9. Regarding Glasgow Coma scale in pediatrics, all the following are true EXCEPT
in modified type it uses 15 score points
it has 3 components
E. enquireabout
timing meal
ofthelast
assessment
arepartoftertiary illchildEXCEPT
ofcritically
11Allthefollowing
A. coagulationprofile
8. renal function
C. echocardiography
D arterial and venous catheters
E. lumbar puncture
andsweetsin playroom,
with candies
toddlerwasplaying
13.A 2-yearold he
anattackof wheezing
developed of breath,the parentsbroughtthe
andshortness
room,RRwas68/minwithsubcostal
childto emergency and02saturation
retractions,
was 92%.
Of the following, the MOSTproper next action is to
A. arrange for urgent bronchoscopy
B. arrange for bedside CXR
C. arrange for CT chest
D. encourage coughing
E. reassure the parents
14. You received a call from your neighbor, he had a 12-month-old baby boy who
suddenly chock with a candy. When you arrive you find the baby unconscious.
Of the following, the NEXT proper action is to
open airway and give rescue breathing
give 5 back blows
wpow?
15.All
give 5 chest thrusts
a series of 5 back blows and chest thrusts
perform endotracheal intubation
100
16. All the following factors are attributing in definition of clinically significant
bradycardia EXCEPT
A. heart rate
8. temperature
C. perfusion
0. mental status
E. blood pressure
18. You are evaluating a 3-year-old conscious child with bradycardia and poor perfusion
in the emergency department.
Of the following, the MOST important first step in treatment is
A. maintaining adequate perfusion by chest compression
B. maintaining the airways and assisted breathing
C. treating precipitating factors as hypoglycemia
D. epinephrine therapy
E. atropine therapy
F909"?absence of P wave
poor perfusion
constant RR
21. You were engaged in CPRfor 3-year-old child found unresponsivein the ward. You
were performingcyclesof 15 chest compressions
and 2 breathingsalternativelywith
101
I"
a-
anothermedical
showsPEA(pulseless
electrical
activity)
Of the following,the correctNEXTactionisto
A. give a shockof 0.5 joule/kg
B. give a shockof 1 joule/ kg
C. give a shockof 2 joules/kg
D. give adrenaline0.01mg/kgiv
E. give adrenaline0.1mg/kg iv
youevaluatethe pulsewh.
After2 minof resuscitation
personal.
TheAEDalready
status. isattached.
*le
-v
\3,
22. Youwere engagedin CPRfor 3-year-oldchildfound unresponsivein the ward. You i
mph???
systolic blood pressure minus intracranial pressure
mean blood pressure minus lntracranial pressure
mean blood pressure
l
mean blood pressure plus CSF pressure
24. All the following are recognized brain herniation due to increase intracranial
pressure EXCEPT
A. cingulate
B cerebraltonsillar
C. transcalvrial
D. uncal
E. upward cerebellar
l
of traumaticbraininjury(TBI),the childin this
the classification
injury.Regarding
scenario has
A. mild TBI
8. moderate TBl
C. severe TBl
102
D. pure axonal brain injury
E. parenchymal brain hemorrhage
26. All the following are signs of increased intracranial pressure (ICP) and impending
brain herniation EXCEPT
A. pupillary dilation
B. 4h cranial nerve palsy
C. systemic hypertension
D. bradycardia
E. extensor posturing
27. All the following are regarded as first tier therapy of increased intracranial pressure
EXCEPT
A. intubation
B. controlled mechanical ventilation
C. head of bed elevation
D. sedation and analgesia
E. barbiturate infusion
28. All the following are important to diagnose brain death status in a child EXCEPT
deep coma of unknown cause
apnea
29. All the following are red flags in evaluating a patient with syncope EXCEPT
syncope with exercise
family history of syncope
30. An 11-year-oid adolescent boy came to your clinic with a complaint of been
withhotwaterafterhefeltdizzy.ratherconfirms
whilebathing
collapsed thattheboy
was extremely pale when founded. He added that he take few minutes to recover, also
he had past history 01 similar condition one month ago when he was urinating.
Of the following, the MOST likely explanation for this condition is
long QT syndrome
POP.)
hypertrophic cardiomyopathy
neurocardiogenic syncope J
drug abuse k
103
E. seizure disorder
31. Of the following, the mandatory test/study for all patients presenting for the first
time with syncope is
A. ECG
B. EEG
C. echocardiography
D. holter monitoring
E. complete blood count
33. You are treating an 8-month-old baby in intensive care unit with cold shock. In the
first 15 min he received adequate fluid therapy and a colleague had started dopamine
for him. The baby condition is still unresponsive.
0f the following, the BEST option now is to start
epinephrine
hydrocortisone
@905? norepinephrine
terlipressin
angiotensin
34. Septic shock usually shows a combination of hypovolemic and distributive shock in
addition to
A. obstructive shock
B. cardiogenic shock
C. acute respiratory distress syndrome
D. disseminated intravascular coagulation
E. decreased synthesis
steroia
35. The percentageof Fiozthat canbe deliveredvia the nasalcannulais up to
w%
40%
p.009"?50%
60%
80% \
104
36. The percentage oi Flo, that can be delivered via a mask is up to
A, 35%
I, 45%
C, 65%
D, 75%
E, 85%
gnpnu
. encephalitis
CNS stimulants
heart failure
38, All the following are controlled mode of mechanical ventilation EXCEPT
A. pressure support ventilation (PSV)
3, conventional mechanical ventilation (CMV)
C. intermittent mechanical ventilation (IMV)
D. synchronized Intermittent mechanical ventilation (SIMV)
E. synchronized intermittent positive pressure ventilation (SIPPV)
39' A thaw-old fullterm baby delivered to a mother with gestational diabetes by CS,
he developed an increasing respiratory distress and grunting with blood gases values
indicative of respiratory failure.
Of the following, the BEST mode to be used in mechanical ventilation is
A. intermittent mechanical ventilation (NV)
8. synchronized intermittent mechanical ventilation (SIMV)
C. synchronized intermittent positive pressure ventilation (SIPPV)
D, pressure-regulated volume control (PRVC)
E. pressure support ventilation (PSV)
41. All the following are recognized complications of mechanical ventilation which can
result from use of high tidal volume and/or inspiratorypressure EXCEPT
A, voiutrauma
105
barotrauma
{"999
oxytrauma
decrease surfactant production
atelectotrauma
42. You are ventilating a fullterm baby with meconium aspiration syndrome, he was
fine on the machine, suddenly the monitor shows severe drop in 02 saturation. You
checked the ventilator; it was working well with its connections.
Of the following, the MOST likely cause is
A. 02 source supply
8. endotracheal tube blockade
C. self extubation
D. low inspiratory pressure value
E. low PEEP value
44. Prior to extubation and in order to reduce airway narrowing after extubation it is
advised to use
dexamethasone IV
betamethasone IV
45.
"'90P?inhaled budesonide
nebulized budesonide
hydrocortisone lV
91.0.0???
elevation of the head of the bed to 30 degrees
use of a protocolfor oral decontamination
liberal use of effective antibiotics
frequent endotrachealtube suction
46. In survivingdrowningpatients;expectingbrainedemacanoccurwithin
A. 5 min
106
15 min
wpow
30 min
60 min
several hours
47. Of the following, the MOST common factor that increasesthe likelihood of
drowning in adolescent (USA) is
A. epilepsy
8. long QT syndrome
C. alcohol
D. myocarditis
E. external trauma
49. All the following are component of cold water shock resulted from immersion in
cold water EXCEPT
A. hypoventilation
B. decrease breath holding ability
C. hypertension
D. ectopics
E. SVT
50. You are treating a 6~yearold child who survived drowning after prolonged CPR, the
mother is asking about the possibility of full neurological recovery of her child.
Of the following, the BEST response is that it is possible if he regained full
consciousness within
A. few hours
B. 12 hours
C. 24 hours
D. 48 hours
E. 72 hours
107
cutey C i
ANSWERS
WW
1.(A). Fever is the most common reason for a sick child visit. Most fevers are the result
of self-limited viral infections. However, pediatricians need to be aware of the age-
dependent potential for serious bacterial infections (e.g., urinary tract infections,
sepsis, meningitis, pneumonia, dysentery, osteoartlcular infection).
2.(B).
3.(A). A child found unresponsive from an unwitnessedcollapse should be approached
with a gentle touch and the verbal question, "Are you OK? If there is no response, the
caregiver should immediately shout for help and send someone to both activate the
emergency response system (EMS) and locate an automated external defibrillator
(AED).
4.(B). The Depth of chest compression should be at least 1/3 anterior-posterior
diameter of chest, about 1 1/2 inches (4 cm) in infants and 2 inches (5 cm) in children.
5.(B). After activation of EMS the next step is to check for pulse and find definite pulse
within 10 seconds.
6.(B). In summary, the plan of action for an unwitnessed infant found unresponsive as
follows
Activate EMS and ask for AED machine.
" Check for definite pulse, if found
give one breath every 3 seconds.
Recheck pulse if it is < 60/min add chest compressions.
In case of no pulse found start directly chest compressions, for one rescuer give 30
compressions with 2 breaths and for 2 rescuers give 15 compressions and 2 breaths,
reassess every 2 minutes.
* Check for the rhythm if it is shockable or not, if it is shockable give one shock and
continue CPR.
Continue CPR until health care provider comes or the victim moves.
7.(C). For a neonate 260 mm Hg, for an infant it is 270 mm Hg and for an older child it is
290 mm Hg, definitely the adults value is inapplicablein children.
8.(C).
9.(C). Although the GCS has not been validated as a prognosticscoring system for
infants and young children as it has been in adults, it is commonly used in the
assessment of pediatric patients with an altered level of consciousness. The GCS is the
most widely used method of evaluating a childs neurologic function and has 3
components. Individual scores for eye opening, verbal response, and motor response
are added together, with a maximum of 15 points. Patients with a GCS score s8 require
aggressive management, including stabilization of the airway and breathing with
108
endotracheal intubation and mechanical ventilation, respectively, and, if indicated,
placement of an intracranial pressure monitoring device.
10.(D). The D means Disability which refers to assessment of the childs neurologic
function in terms of the level of consciousness and cortical function. It can be assessed
quickly by checking the pupilary light respose and using Glasgow Coma scale, so the
response D is only partially true. The causes of decreased level of consciousness in
children are numerous and include conditions as diverse as respiratory failure with
hypoxia or hypercarbia, hypoglycemia, poisonings or drug overdose, trauma, seizures,
infection, and shock. All that system is represent the primary assessment of critically ill
child.
11.(A). The components of a secondary assessment include a focused history and
focused physical exam. The history should be targeted to information that could
explain cardiorespiratory or neurologic dysfunction and should take the form of a
SAMPLE history (Signs/symptoms, Allergies, Medications, past medical history, timing
of Last meal, and Events leading to this situation). Medical personnel not engaged in
resuscitative efforts can be dispatched to elicit history from witnesses or relatives. The
physical exam during the secondary assessment is a thorough head-to-toe exam,
although the severity of the childs illness or injury could necessitate curtailing portions
of the exam or postponing nonessential elements until a later time.
12.(E). The tertiary assessment occurs in a hospital setting, where ancillary laboratory
and radiographic assessments contribute to a thorough understanding of the childs
condition. A basic blood chemistry profile, complete blood count, liver function tests,
coagulation studies, and arterial blood gas analyses give fairly broad (but somewhat
nonspecific) estimates of renal function, acidbase balance, cardiorespiratory function,
and presence or absence of shock. Chest radiographs can be useful to evaluate both
the heart and lungs, although more detailed estimates of heart function and cardiac
output can be made. Choice E is not part of assessment.
13.(A). A history consistent with foreign-body aspiration is considered diagnostic. Any
child in the proper setting with the sudden onset of choking, stridor, or wheezing has
foreign body aspiration until proven otherwise. The choice A is the best answer as the
child start to show signs of respiratory distress with borderline 02 sat. The choice B is
the next appropriate action while encourage coughing may be appropriate at home
when no respiratory distress is there.
14.(A). In unconscious child, the child should be gently placed on the ground, supine,
and then the provider should open the airway with the head-tilt/chin-lift maneuver and
attempt mouth-to-mouth ventilation. If ventilation is unsuccessful, the airway is
repositioned, and ventilation attempted again. If there is still no chest rise, attempts to
remove a foreign body are indicated. In an infant <1 yr old, a combination of 5 back
blows and 5 Chest thrusts is administered, After each cycle of back blows and chest
thrusts, the childs mouth should be visually inspected for the presence of the foreign
body. If identified within fingers reach, it should be removed with a gentle finger
sweep. If no foreign body is visual, ventilation is again attempted. If this is unsuccessful,
109
the head is repositioned, and ventilation attempted again. If there is no chest rise, the
series of back blows and chest thrusts is repeated. For a conscious child >1 yr old,
providers should give a series of 5 abdominal thrusts (Heimlich maneuver) with the
child standing or sitting; this should occur with the child lying down if unconscious.
15.(C). In settings of known complete airway obstruction, endotracheal intubation
should be avoided, and emergency cricothyroidotomy performed instead.
16.(B). A clinically significant bradycardia occurs when the heart rate is slow and there
are signs of systemic hypoperfusion (i.e., pallor, altered mental status, hypotension,
and acidosis). Symptomatic bradycardia occurs most often in the setting of hypoxia but
can also be caused by hypoglycemia, hypocalcemia, other electrolyte abnormalities,
and intracranial hypertension.
17.(A).
18.(B). Alleviating respiratory compromise and maintaining adequate breathing and
oxygenation may correct clinically significant bradycardia. Start monitoring and obtain
IV access. If bradycardia persist it is the time for chest compressions and if continues
you need to start drug therapy. During resuscitation correct possible underlying causes
referred collectively as the 6 H5 (hypoxia, hypovolemia, hydrogen ions
[acidosis],hypokalemia or hyperkalemia, hypoglycemia, hypothermia), and 5 Ts (toxins,
tamponade, tension pneumothorax, thrombosis [in either the pulmonary or cardiac
circulations], and trauma [causing hypovolemia, intracranial hypertension, cardiac
compromise or tamponade])
19.(A). Atropine is indicated in the case of increased vagal tone (e.g., in the setting of
head injury with raised intracranial pressure) or primary atrioventricular block.
20.(D). In sinus tachycardia, the history and onset are consistent with a known cause of
tachycardia, such as fever or dehydration and P waves are consistently present, are of
normal morphology, and occur at a rate that varies somewhat. ln SVT, onset is often
abrupt without prodrome and P waves are absent or polymorphic, and when present,
their rate is often fairly steady at or above 220 beats/min. Poor perfusion can present
in either causes as in dehydration with tachycardia and in late stage of SW.
21.(D). Pulseless electrical activity (PEA) is a clinical condition characterized by
unresponsiveness and lack of palpable pulse in the presence of organized cardiac
electrical activity. In this situation (and in asystole) adrenaline is indicated in cycles of 3.
5 min, considering an advanced airways and reevaluation for the pulse status; if it
became shockable give shock of 2 joules/kg. You need to look also for correctable
causes (the 6H and ST). If it became normal that goes for (ROSC) post-cardiac arrest
care.
110
24.(B). In fact there are 6 types of brain herniation in addition to what have been
mentioned in the question plus cerebellar tonsils and cerebral herniation through
foramen magnum.
25.(C). The key for severity of TBI is GCS score, patient with GCS of 3-8 regarded as
severe type while those between 9-12 regarded as moderate.
26.(B). The development of increased ICP with impending herniation may be heralded
by new onset or worsening headache, depressed level of consciousness, vital sign
changes (hypertension, bradycardia, irregular respirations), and signs of 6th (lateral
rectus palsy) or 3rd (anisocoria [dilated pupil], ptosis, down-and-out position of globe
as a result of rectus muscle palsies) cranial nerve compression.
27.(E). The first tier therapy also include neuromuscular blockade, CSF drainage, and
the use of osmolar agents as hypertonic saline (3%) and mannitol. 2" tier therapy
include barbiturate infusion, decompressive craniectomy, mild hypothermia
hyperventilation and lumbar CSF drainage.
28.(A).
29.(C). Typical prodromal symptoms prior to syncope include light headedness,
dizziness, and nausea, sweating, and feeling hot or cold. Patients may report visual field
changes and rushing in their ears.
30.(C). Typically, the patient with syncope will have been standing for a period of time,
often on a hot day, or has gotten up suddenly from sleep or resting in a supine position.
Occurrence in the shower is common, presumably caused by standing and vasodilation
caused by hot water. For boys, the occurrence while urinating while standing is
sometimes reported. The occurrence of syncope in girls while sitting or standing and
having their hair brushed is common.
31.(A). All patients presenting with a first episode of syncope should have an
electrocardiogram obtained, looking primarily for QT interval prolongation,
preexcitation, ventricular hypertrophy, T-wave abnormalities, and conduction
abnormalities. Other tests may be needed depending on the results of the initial
evaluation.
32.(B). It represent a decreased in cardiac output secondary to direct impediment to
right- or left-heart outflow or restriction of all cardiac chambers.
33.(A). After 15 min of treating shock, inotropes like dopamin need to be started, if the
shock still unresponsive epinephrine for cold and norepinephrine for warm shock
should be titrated. Hydrocortisone is considered after 60 min (Catecholamine resistant
shock). Other options are used in warm shock and usually after other options are
exhausted.
3MB). Options C and D may be part of pathophysiology of septic shock, it is not a type.
35.(B). The typical Fioz value using this method is between 23% and 40%, and can be
measured with following formula
Fioz%Ozdelivered = 21 %+( nasal cannula flow (L/min) X3), and the flow rate should be
<5 L/min.
111
36.(C). Oxygen flow rates vary from 510 L/min, yielding typical Fioz values between
0.30 and 0.65.
37.(E). Causes rapid shallow breathing.
38.(A). Except the option A all other forms of mechanical ventilation variables are
controlled either fully by the machine as in options B and C which need to paralyze the
patient to get the synchrony or partially as in options D and E where there is patient
trigger by an effort to respiration followed by the machine active work to do the preset
ventilation values; ventilatorpatient asynchrony can occur in this mode also because
tidal volume, inflation pressure, and inspiratory time are determined by the ventilator
alone. PSV allows the patient to control as much of the rate, tidal volume, and
inspiratory time as much as possible. It is considered a gentler form of mechanical
ventilation and is designed for patients with relatively minor lung disease or for those
with neuromuscular weakness.
39.(D). lMV is not the choice because you need to paralyze the patient; options 8 and C
both are good choice as they provide synchrony with the patient and can achieve the
goal of ventilation by either controlling the pressure or volume values but in presence
the option D in your ventilator, making it the best because it will reduces both baro and
volutrauma.
40.(C). It can also reduce atelectotrauma.
41.(E). It can result from low PEEP.
42.(B). All the given options are true causes for sudden drop of 02. The possible causes
of desaturating baby on ventilator are; Oxygen supply, machine and it is connections,
settings, tube block, self extubation and the patient himself. In the given scenario, the
most likely cause is B because of meconium aspiration. The best action to such scenario
is to disconnect the baby from the machine and use Ambu bagging gently to check the
air entry and rise of Ozsaturation. if no rise you need to change the tube. In case of
unequal air entry, pneumothorax is another possibility.
43.(D). In absence of tachypnea, increased work of breathing, hypoxemia, hypercapnia,
acidosis, diaphoresis, tachycardia, and hypotension and when the ventilator rate is <5
breaths/min or (between 5-10 cm H20 in pressure controlled devices) indicate that the
contribution of the ventilation to minute ventilation is minimal and the baby is ready to
be extubated.
44.(A). Administration of intravenous dexamethasone 0.5 mg/kg every 6 hr for 4 doses
prior to extubation has been shown to minimize the incidence of postextubation airway
obstruction. In patients in whom postextubation airway obstruction develops, the need
for re-intubation may be obviated by administration of nebulized racemic epinephrine
and heliox.
45.(A). The options 8 and C are a true technique, but the most effective strategy to
minimize VAP is regular assessment of extubation readiness and liberation from
mechanical ventilation as soon as clinically possible.
46.(E). Several hours after cardiopulmonary arrest, cerebral edema may occur,
although the mechanism is not entirely clear.
112
' 47.(C). The use of alcohol and drugs greatly increasesthe risk of drowning in teenagers
and adults who die, 30-40%have positive blood alcohol level.
48.(E).
49.(A). Immersion in cold water has immediate respiratory and cardiovascular effects.
Victims experience cold water shock, a dynamic series of cardiorespiratory physiologic
responses that can cause drowning. In adults, immersion in icy water results in intense
involuntary reflex hyperventilation and to a decrease in breath-holding ability to <10
sec, which leads to fluid aspiration. Severe bradycardia, the diving reflex, occurs in
adults but is transient and rapidly followed by supraventricular and ectopic tachycardia
and hypertension. There is no evidence that the diving reflex has any protective effect.
50.(E). Neurologic examination and progression during the lst 24-72 hr are the best
prognosticators of long-term CNS outcome. Children, who regain consciousness within
48-72 hr, even after prolonged resuscitation, are unlikely to have serious neurologic
sequelae.
113
Human Genetics
QUESTIONS
.
kHMED TAWFI
1.When isat-risk
anindividual andnotclinically
genotype express this
thecondition;
phenomena is called
A. geneticimprinting
B. genetic anticipation
C. uniparentaldiasomy
D. non expressiveness
E. non penetrance
FPOP?diagnostic
pharmacogentic
linkage testing
testing
testing
114
homocysteine level
POE?
prothrombin test
factor V lelden
MTHFR DNA testing
E" folate level
990?
performing appropriate genetic testing
refer to support groups
talking about rewarding gene therapy
8. Enzyme replacement therapy are available for the following disorders EXCEPT
A. Woiman disease
8. Gaucher disease
C. Fabry disease
D. Mucopolysaccharidosis type Vi
E. Pompe disease
115
forgenetic
tool
screening IS
disorders
11.The
MOST
important
genetic study
family history
WP??? karyotyping
prenatal diagnosis
preimplantation genetic screen
isexplained
12.Oneofthefollowing inheritance
bydigenetic
vitamin D dependent rickets
retinitis pigmentosa
13. Parent-to-child
transmission Is a characterof autosomal
(verticaltransmission)
dominantinheritance,for manypatientswithan autosomaldominantdisorderthereis
no history of an affected family member.
All the following are true explanations EXCEPT
variable expressiveness
new mutation
14.
@905? somatic mutations (mosaicism)
digenetic inheritance
incomplete penetrance
990???
male to male transmission
males and females are equally affected
skipped generation
both parentscarriedthe affected gene
POP.)
E
males and females are equally affected
consanguinity plays important role
25% recurrence risk
116
6-8%
mph?
9-11%
12-14%
15-17%
18. In genetic disorders, male to male transmission occurs in Y- linked inheritance and
in
A. X- linked inheritance
B. pseudodominant inheritance
C. digenetic inheritance
D. autosomal dominant disorder
E. pseudogenetic inheritance
117
The
22. represent EXCEPT
inheritance
anon-traditional
following
A. mitochondrialdisorders
diseases
8. tripletrepeatexpansion
C. imprintingdefects
D. familialclustering
E. uniparentaldisomy
23.The areatriplet
following expansion
repeat EXCEPT
disorders
A. fragile X syndrome
B. myotonic dystrophy
C. Huntington disease
D. spinocerebellar ataxias
E. Kearns-Sayre syndrome
ofincreasing
24.Theobservation severity andearlyageofonsetin
ofthedisease
subsequent generation is called
A. genetic imprinting
B. genetic anticipation
C. uniparental diasomy
D. non expressiveness
E. non penetrance
26. Thefollowinggeneticscenarios
arepossible
to explainAngelmansyndromeEXCEPT
A. inheriting both chromosomes15 from the father
deletion of the maternal 15q12
W90?
mutation in an imprinted gene
mutation in the imprinting center
genetic anticipation
27.TheMOSTcommongeneticcauseof Prader-Willi
syndromeis
inheritingbothchromosomes
15 fromthe mother
deletion of the maternal 15q12
118
E. mutation in the imprinting center
mph?
5-696
7-896
940%
31. A 17-year-old adolescent female with trisomy 21 became pregnant, the chance of
affection of her offspring with the same problem is
A. Not possible
8. 25%
C. 50%
D. 75%
E. 100%
32. All the following are recognized gastrointestinal features of Down syndrome in
neonatal period EXCEPT
A. annular pancreas
B. tracheoesophageal fistula
C. diaphragmatic hernia
D. imperforate anus
E. neonatal cholestasis
33. The MOST important reason for chromosomal analysis in persons suspected of
having Down syndrome is to
119
A. confirm clinicaldiagnosis
B. detect mosaicism
C. reassure the family
D. detect translocations
E. detect rare casesof ring chromosome
34.Achromosomalstudy mother
of22-year~old ofababywithDownsyndromet (14;
forthetranslocation.
that;sheisthecarrier
21)shows thatthe
Youexplained
recurrencerate will be approximately
A. 24%
8. 5-796
C. 8-1096
D. 11-13%
E. 33%
36. You are explaining the motor development to a mother with a baby with Down
syndrome, you told her that there is a wide range of time to achieve his milestone and
as example the baby can walk between
A. 1018 mo
8. 12-24 mo
C. 12-45 mo
0. 15-50 mo
E 18-60 mo
120
0. send for radiological assessment of the airway
E. send for thyroid function test
38. Quad screen is the four maternal serum tests that should be done for all pregnant
women in second trimester as a screening for Down syndrome, which includes the
following tests EXCEPT
A. free B-human chorionic gonadotropin
B. unconjugated estriol
C. androsterone
D. inhibin
E. a-fetoprotein
39. All the following are features of Edwards syndrome (Trisomy 18) EXCEPT
A. low birth weight
B. closed fists
C. cardiac malformations
D. short sternum
E. slopping forehead
40. All the following are features of Patau syndrome (Trisomy 13) EXCEPT
A. scalp defects
3. corneal abnormalities
C. holoprosencephaly
D. capillary hemangiomas
E. prominent occiput
41. Robertsonian translocations involve 2 acrocentric chromosomes that fuse near the
centromeric region with a subsequent loss of the short arms. It can occur in the
following chromosomes EXCEPT
A. 13
B. 15
C. 17
D. 21
E. 22
42. One of the common chromosomal deletions which can lead to retinoblastoma is
A. 4p-
3. 5p-
C. 9p-
D. 13q-
E. 18p-
121
ofmicrodeletion
areexamples
43.Allthefollowing EXCEPT
syndromes
A. Williams
B. Prader-Willl
C. Angelman
D. DiGeorge
E. Wolf-Hirschhorn
asTurnersyndrome
femalechildhadbeendiagnosed
44.An18-month-old (45,x0),
FISHanalysisshowsY chromosomemosaicism.
Of the following, the MOST appropriate next step is to
A. refer to endocrinologist
reassure as the course will be milder
990!
advise for feminizing hormones
arrange for laparoscopic removal of gonads
rear the child as male gender
45. All the following are recognized features of Turner syndrome EXCEPT
female phenotype
male phenotype
WP???hypothyroidism
type I diabetes mellitus
gonadal dysgenesis
46. All the following are characteristic features of fragile X chromosome of a 3-year-old
male child EXCEPT
A. intellectual disability
8. autistic behavior
C. macroorchidism
D. hyperextensible finger joints
E. characteristic facial features
122
""""' W , , W
""""""
NPR
Human Genetics
ANSWERS
)AHMED TAWFIQ
1.(E).
2.(B).
3.(C). In fact, all the mentioned explanations about the disease are true but it is
generally agreed that predictive genetic tests should be performed for children if the
results of the test will benefit the medical management of the child. Otherwise, the test
should be deferred until the child has an understanding of the risks and benefits of
testing and can provide informed consent.
4.(B). In a disease cause by many genes and affected by environmental factors,
controlling of environmental factors may reduce disease progression as in cigarette
smoking and ulantitrypsin deficiency, familial breast cancer and mammography.
S.(D). Knowledge of individual genotypes (pharmacogentic genetic testing) will guide
pharmacologic therapy, allowing customization of choice of drug and dosage to avoid
toxicity and provide a therapeutic response. An example of this is testing for
polymorphisms within the methylenetetrahydrofolate reductase (MTHFR) gene for
susceptibility of potentially increased toxicity to methotrexate antimetabolite therapy
for treatment of acute lymphoblastic leukemia.
6.(B). Maternal age > or equal to 35 is an indication for genetic counseling. Genetic
counseling is a communication process in which the genetic contribution to health is
explained, along with specific risks of transmission of a trait and options to manage the
condition and its inheritance. The counselor is expected to present information in a
neutral, nondirective manner and to provide support to the individual and family to
cope with decisions that are made.
7.(E). Gene therapy still experimental and rewarding therapy is still a hope.
8.(A).
S.(A).
10.(A).
11.(B). The family history remains the most important screening tool for pediatricians
in identifying a patients risk for developing a wide range of diseases, from
multifactorial conditions, such as diabetes and attention-deficit disorder, to single-gene
disorders such as sickle cell anemia and cystic fibrosis.
11(8). Digenic inheritance explains the occurrence of retinitis pigmentosa (RP) in
children of parents who each carry a mutation in a different RP-associated gene. Both
parents have normal vision, as would be expected, but their offspring who are double
heterozygoteshaving inherited both mutationsdevelop RP. Digenic pedigrees can
exhibit characteristics of both autosomal dominant (vertical transmission) and
autosomal recessive inheritance (1 in 4 recurrence risk).
123
13.(D). Double heterozygotes
14.(E). Autosomal dominant inheritance is determined by the presence of 1 abnormaI
gene on 1 of the autosomes (chromosomes 122). Autosomal genes exist in pairs, with
each parent contributing 1 copy. In an autosomal dominant trait, a change in 1 of the
paired genes has an effect on the phenotype.
15.(A). Horizontal transmission is the characteristic feature of autosomal recessive
inheritance, the observation of multiple affected members of kindred in the same
generation, but no affected family members in other generations.
16(8). The risk of a genetic disorder for the offspring of a first-cousin marriage (68%)
is about double the risk in the general population (34%).
17.(C). Pseudodominant inheritance refers to the observation of apparent dominant
(parent to child) transmission of a known autosomal recessive disorder. This occurs
when a homozygous affected individual has a partner who is a heterozygous carrier,
and it is most likely to occur for relatively common traits, such as sickle cell anemia.
18.(D).
19.(D).
20.(C). Mitochondrial inheritance represents a nontraditional inheritance (not follow
the usual Mendelian inheritance), both sexes can be affected from the affected
mitochondrial DNA genome in the ovum (sperm contain few mitochondria which
finishes during fertilization), so no male to offspring transmission.
21.(C). The mitochondria are the cells suppliers of energy, so the organs that are most
affected by the presence ofabnormal mitochondria are those that have the greatest
energyrequirements, such as the brain, muscle, heart, and liver. Common
manifestations include deve|0pmenta|delay, seizures, cardiac dysfunction, decreased
muscle strengthand tone, and hearing and vision problems. Examples of
mitochondrialdisorders include MELAS (myopathy, encephalopathy, lactic acidosis,
andstrokelike episodes), MERRF(myoclonic epilepsy associated with ragged red fibers),
and Kearns-Sayre syndrome (ophthalmoplegia, pigmentary retinopathy, and
cardiomyopathy).
22.(D). The first three represent the non-traditional inheritance; familial clustering
represents a form of pseudogenetic transmission, while uniparental disomy is a type of
imprinting defects.
23.(E). Represent mitochondrial disorder.
24.(B).
25.(C). Several studies suggest that there may be a small but significantly increased
incidence of imprinting disorders, specifically Beckwith-Wiedemann and Angelman
syndrome, associated with assisted reproductive technologies such as in vitro
fertilization and intracytoplasmicsperm injection. However, the overall incidenceof
these disorders in children conceived using assisted reproductive technologies is likely
to be <1%.
26.(E). The first statement is called uniparental disomy when both copies of a
chromosome is inherited from one parent, the second call genetic imprinting when one
124
gene copy is silenced in one side; either in mother or father resulting in each situation
in different syndromes, third and fourth distracters is possible but are uncommon
causes.
27.(C). in about 70% of cases missing paternal 15q12 lead to what is called genomic
imprinting. The same clinical effect if the child inherted the same chromosome (15)
from the mother (25-29%). In Angelman syndrome, paternal UPD of chromosome 15 is
rarer and is observed in approximately 5% of the cases (missing the maternal
chromosome 15).
28.(C). Some multifactorial inherited disordershave a sex predilection, as indicated by
an unequal male: female incidence. Pyloric stenosis, for example, is more common in
males, whereas congenital dislocation of the hips is more common in females. Where
there is an altered sex ratio, the risk is higher for the relatives of an index case whose
gender is less commonly affected than relatives of an index case of the more commonly
affected gender. For example, the risk to the son of an affected female with infantile
pyloric stenosis is 18%, compared with the 5% risk for the son of an affected male. An
affected female presumably has a greater genetic susceptibility, which she can then
pass on to her offspring.
29.(A). The specific indications for studies include advanced maternal age (>35 yr) or
multiple abnormalities on fetal ultrasound (prenatal testing), multiple congenital
anomalies, unexplained growth restriction in the fetus or postnatal problems in growth
and development, ambiguous genitalia, unexplained intellectual disability with or
without associated anatomic abnormalities, primary amenorrhea or infertility,
recurrent miscarriages (23) or prior history of stillbirths and neonatal deaths, a 15t-
degree relative with a known or suspected structural chromosome abnormality, clinical
findings consistent with a known anomaly, some malignancies, and chromosome
breakage syndromes (e.g., Bloom syndrome, Fanconi anemia).
30.(A). Clinical cytogenetics is the study of chromosomes: their structure, function,
inheritance, and abnormalities. Chromosome abnormalities are very common and
occur in approximately 1-2% of live births, 5% of stillbirths, and 50% of early fetal losses
in the lst trimester of pregnancy.
31.(C). Most males with Down syndrome are sterile, but some females have been able
to reproduce, with a 50% chance of having trisomy 21 pregnancies.
32.(C). It also includes duodenal atresia and Hirschsprung disease.
33.(D). Chromosome analysis is indicated in every person suspected of having Down
syndrome. If a translocation is identified, parental chromosome studies must be
performed to determine whether one of the parents is a translocation carrier, which
carries a high recurrence risk for having another affected child.
34.(B). Translocation (21; 21) carriers have a 100% recurrence risk for a chromosomally
abnormal child, and other Robertsonian translocations, such as t(14;21), have a 5-7%
recurrence risk when transmitted by females.
35.(E). Developmental delay is universal. Cognitive impairment does not uniformly
affect all areas of development. Social development is relatively spared, but children
125
with Down syndrome have considerable difficulty using expressive language.
Understanding these individual developmental strengths will maximize the educational
process for children with Down syndrome. Persons with Down syndrome often benefit
from programs aimed at stimulation, development, and education. These programs are
most effective in addressing social skills that often appear advanced for the intellectual
delay.
36.(C).
37.(B). The incidence of atlantoaxial sublaxation or instability in Down syndrome is
about 10-30%, affected patient should not allowed to participate in high risk sports as
include diving starts in swimming, butterfly stroke, diving, pentathlon, high jump,
equestrian sports, gymnastics, football, soccer, alpine skinning and warm up exercises
placing stress on the head and neck
38.(C). Androsterone is generally considered to be an inactive metabolite of
testosterone, and it is not related to quad test.
39.(E). Slopping forehead is a feature of Trisomy 13.
40.(E). Prominent occiput is a feature of Edwards's syndrome.
41.(C). It includes also chromosome number 14.
42.(D).
43.(E). Microdeletions involve loss of small chromosome regions so that the affected
individuals can have a distinctive phenotype depending on the number of genes
involved. All the mentioned syndromes are common microdeletions and the board
candidate should be familial with their phenotypic descriptions and clinical
manifestations. Wolf-Hirschhorn syndrome is chromosomal deletion syndrome.
44(0). Phenotypic females with 45, X/46, XY mosaicism have a 15-30% risk of
developing gonadoblastoma. The American Academy of Pediatrics has recommended
the use of FISH analysis to look for Y-chromosome mosaicism in all 45, X patients. If Y
chromosome material is identified, laparoscopic gonadectomy is recommended.
45.(D). Male phenotype can occur with mosaic type of Turner syndrome, and type II
insulin resistance is the true answer.
46.(C). The macroorchidism may not be evident until puberty. The facial features,
which include a long face, large ears, and a prominent square jaw, become more
obvious with age.
126
88 OIC ISO ers
QUESHONS
MW
1. Inborn
errors biochemical
(IEM)arehereditary
of metabolism caused
disorders by
single-genemutations that result in alteration of
A. chromosomal number or structure
mitochondrial synthesis or function
primaryfat structureor the amountof fat synthesized
FPO? carbohydrate
primary
or theamountof proteinsynthesized
primaryproteinstructure
ofcarbohydrate
ortheamount
structure synthesized
biochemical
(IEM)are hereditary
2. Inbornerrorsof metabolism causedby
disorders
single-gene mutations that encode specificproteins.
The following are the common characteristicsof IEM EXCEPT
A. the majority of conditionsare inherited as autosomalrecessive
B. the laterthe appearance the moresevereis the disease
of clinicalsymptoms,
C. early diagnosisis of paramountimportancebefore irreversibleorgandamage
D. the affected infant is normal at birth and becomessymptomaticlater on in life
E. there is a variation in the severity of the phenotype based on the gene
mutation
3. All the following are widely used modalities for treatment of inborn errors of
metabolism (IEM) EXCEPT
A. special diets
B. gene therapy
C. peritoneal dialysis
D. liver transplantation
E. administration of the deficient metabolite
4. A 28-day-old girl presented with lethargy, poor feeding, and repeated vomiting for
last 5 days;IV fluid and empiricalantibioticswere started; later she developsrepeated
seizuresnot respondingto IV calcium,glucose,36, and anticonvulsantdrugs. Septic
screenis negative;serum ammonia is elevated with normal anion gap and normal pH.
Family history reveals 2 siblingsdied with same scenario.
Of the following, the MOST likely diagnosis is
A. galactosemia
B. hyperglycinemia
C. organic acidemias
D. phenylketonurea
127
E. urea cycledefects
5 You ametabolic
suspect ina30-d3li'0Id
problem With9007
8" presented feeding,
and
lethargy,
vomiting Previous
convulsion. died
sibling With
thesame condition.
Serum
ammonia, andaniongaparenormal.
pH,HC03,
Ofthe following,the MOSTlikelydiagnosisis
A. porphyria
galactosemia
organic acidemia
FPDFPsweaty feet
boiled cabbage
swimming pool
128
9. In the classic phenylketonurea, the affected infant is normal at birth, intellectual
disability develops gradually if the infant remains untreated, and cognitive delay may
not be evident for the first few months.
All the following are true about neonatal screening program of this condition EXCEPT
Guthrie test was the 1 method used
optimal time for Guthrie test at 7-14 of life
W905?
blood phenylalanine
fluorometric
diagnosis
concentration
may rise to a diagnostic level as early as 4 hr after birth
and tandem mass spectrometry give a low false-positive rate
must be confirmed by measurement of plasma phenylalanine
11. A 4-month~old boy presented with failure to thrive, rapid breathing, and repeated
vomiting. On examination, there is hepatomegaly and abdominal sonography revealed
renal calcification.
of the following, the MOST likely urine odor of this infant is
A. mousey
B. rotting fish
C. sweaty feet
D. boiled cabbage
E. swimming pool
12. A 10-month-old boy presented with failure to thrive, fever, jaundice, hepatomegaly,
and severe rickets. Investigations revealed hypoglycemia and normal anion gap
metabolic acidosis.
Of the following, the MOST likely enzyme deficiency is
A. acid B-glucosidase
B. B-hexosaminidases
C. acid sphingomyelinase
D. phenylalanine hydroxylase
E. fumarylacetoacetate hydrolase
129
recurrent
had admissionsbecauseof fever,jaundice
hospital
boy
13.A 15-month-old thereishepatOmegaly.
fits;onexamination,
subcutaneous bleeding,andhypoglycemicpain
legs
severe withretraction
associated ofthe
Inthe lastattack,the c developed
hild
neckandtrunk. is
Ofthe following,the MOST likelydiagnosis
galactosemia
organicacidemia
$90?»
The
14. MOST
ureacycledefect
tyrosinemiatype 1
maplesyrupurinedisease
appropriate thediagnosis
toconfirm
method 1isby
oftyrosinemia
type
elevated level of
A. afetoprotein
plasma tyrosine
heserum methionine
D. serum succinylacetone
acid
E. urinaryS-aminolevulinic
15.A 10-day-old
boypresented poorfeeding,
withlethargy, and
vomiting,
repeated
recurrent
hypoglycemic to IVglucose.
fitsnotresponding was
he
Onexamination,
withsevere
hypertonic withflaccidity.
alternating
opisthotonos
Of the following, the MOSTlikely diagnosisis
tyrocinemia
galactosemia
16.
W909?phenyletonuria
homocystinurea
maple syrup urine disease
130
C. refractive errors
D. foveal hyperplasia
E. lack of binocular vision
18. A 6-year~old tall and thin boy who had failed preparatory exam for schoolentry
found to have myopia and subluxation of the ocular lens.
0f the following, the MOST important challenge is to know whether he is responsive to
folic acid
vitamin C
FLUFF?
vitamin
vitamin
vitamin
E
86
812
19. A 6-year~o|dgirl that looks tall and thin with light skin. On examination she has a
peculiar malar flush, subluxation of the ocular lens, developmental delay, and severe
hypertension.
Of the following,the MOST likely cause of hypertension is
A atherosclerosis
B. hyperthyroidism
C. thromboembolism
D. coarctation of aorta
E. renal artery stenosis
meow
vitamin C
vitamin 86
methionine restriction
21. The diagnosis of homocystinuria is usually made after 3 yr of age when the
ophthalmologist found
A. cataracts
B. glaucoma
C. astigmatism
D. ectopia lentis
E. retinal detachment
22. 11month-old boy presented with repeated convulsions, poor eye contact,
An
exaggerated startle response to noise, and large head. Ophthalmic exam revealed
cherry-red spot.
Of the following, the MOST likely diagnosis is
131
A. Tyrosinemia
B. Fabrydisease
C. Gaucherdisease
disease
D. TaySachs
E. Niemann-pickdisease
isa
following
the
of forboth
feature
characteristic infantile
Sandhoff
disease
One
23
disease
andTay-Sachs
A. splenomegaly
hepatomegaly
cardiacinvolvement
mph? spots
retinalcherry-red
mildbonyabnormalities
mildbruising,
withepistaxis, intermittent
bonepain,
24.A 12-year-old
boypresented
andmassive bloodcountshowsthrombocytopenia
Complete
splenomegaly. andmild
anemia. studyrevealedlyticlesionsand Erlenmeyerflaskdeformityofthe
Radiological
distal femur.
0f the following,the MOST likely diagnosisis
A. Gaucher disease
Sandhoff disease
W90?
Tay-Sachs disease
von Gierke disease
Niemann-pick disease
A9-year-old
25. boyadmint0thehOSpital
because due
ofleftfemur
offracture toa
minor Pasthistoryrevealed recurrent attacks of
trauma. epistaxis and intermittent
generalized
bonepain.Onexamination,
thereismassive
Ofthe the
following,
MOST
appropriate
treatment
Practical ofthis is
disease
splenomegaly.
A- gene therapy
8. livertransplantation
C. enzyme
replacement
substrate
reduction
. bonemarrow agents
2.
transplantation
26. A 2- ea
milestonz,
Sud? present
With
failure
I mmtocomaWith tothrive, of
regression
a
developmentre
On there
examination,
hepatosplenom moderatesurroundings
0fthe
fonowlngeiglv
' e 3:
denOpathy
lympha .
Gaucher yd'agnosm
STlikel
IS
disease
132
D. Von Gierke disease
E. Niemann-pick disease
133
with
nted growth retardation,
hepatomegalyl
reCurrent and
boyprese
32.A2-year-old reveals
Istudy
biochemica blood
elevated'aCtate,
trigl
ofhypoglycemia;
attacks YCeride
anduricacidlevels. EXCEPT
ofthisdisease
lications
arecomp
Allthefollowing
A pancreatitis
B. renal failure
C. cardiomyopathy
D. hepaticadenoma
E. hypertension
pulmonary
withgrowth retardation, recurrent attacksof otitis
girlpresented
33.A3-year-old seizures.Onexamination, there is hepatOmegaly
andhypoglycemic
diarrhea,
media,
count
neutrophil
herabsolute
and is600/uL.
(ANC)
theMOST
Ofthefollowing, likely is
diagnosis
A. Kostmanndisease
8. cyclicneutropenia
neutropenia
C. drug-induced
D. glycogen disease
storage lb
E. Shwachman-Diamond syndrome
91.0.05»
administration of table sugar
continuousnasogastricinfusion of glucose
oral administrationof uncooked corn starch
36. A - oldboy-
presented withpersistent head lag, dimwlt
breathing
rapid
, 2;"?
feeding epatomegaly.ChestX- ray showed cardiomegaly-
0f the followingI the MOST ' ' '
A. liverbiopsy appropriate testIS
confirmatory
5- musclebiopsy
C. electrocardiom
B phy
D. serum creatine
phosphokinase
134
1... w.
:ef nLl'lhlIIv
mpoe>cardiac transplantation
nocturnal ventilatory support
specific enzyme replacement therapy
38. A 28day-old boy presented with prolonged neonatal jaundice, lethargy, poor
feeding, and failure to regain birth weight. On examinationthere are hepatomegaly
and cataract.
Of the following, the MOST likely injured organs in this disease are
A. eyes, liver, and brain
B. kidney, liver, and eyes
C. kidney, liver, and brain
0. kidney, heart , and brain
E. pancreas, liver, and brain
ppm?»renal injury
myocardial damage
intellectual disability
41. A 9~month-old
boywith exclusivebreastfeedingpresentedwith jaundice,vomiting,
lethargy, hepatomegaly, and hypoglycemicfits when sweetened cereal added.
135
. time, hypoalbuminemia,ele
prolongedclotting
showed
rato findings
31d
[bilmbm and
levels,
transaminase tubular
proxrmal ,
dysfunction. . vation of
theMOST
Ofthefollowing, is
diagnosis
likely
A. tyrosinemia
galactosemia
organicacidemias
FPO? maplesyrupurinedisease
hereditaryfructoseintolerance
arecommon
42.Allthefollowing between
features fructose
hereditary intolerance
and
galactosemiaEXCEPT
A. failure to thrive
B impaired renal function
C. impaired hepatic function
D. reducing substance in urine
E. impaired intellectual development
FPO!
fruit juice
table sugar
sweetened cereal
44.Theearliestradiographic
signin Hurlersyndromeis
A . thick ribs
B. thickened calvarium
C. enlarged sella
J-shaped
D. abnormalspacingof teeth
E. premature closure of
lambdoid suture
oldboypresented
a murmur in a withtiredness on .
revea ls
ortlc area;echostudy5howed exertion;
.
cardiac auscu'mt'on
. -
aortlc valve disease.On exammaonhe
136
corneal
facialfeatures,
hadmildcoarse clouding, andmilddysostosis
jointstiffness,
multiplex but normal intelligenceand stature.
of the following, the MOST likely diagnosisis
A. Hurler disease
8. Scheie disease
C. Hunter disease
D. Morquio disease
E. Sanfilippo disease
137
CNS
lnvoIVement
severe With
disproportionate mild
Inmucopolysaccharidosis,
51 to
isunique
features
physical disease
A. Scheie
B. Hurlerdisease
C. Hunterdisease
D. Morquiodisease
E. Sanfilippodisease
manifestations
skeletal
are disease
ofMorqUio EXCEPT
52. All the following
A. kyphosis
B. genuavalga
C. waddlinggait
D. shorttrunkandneck
E. bullet-shapedphalanges
tolife-threatening
issusceptible
mucopolysaccharidoses
ofthefollowing
53.Which
anddislocation?
instability
atlantoaxial
A. Hurler disease
8. Scheie disease
C. Hunter disease
D. Morquio disease
E. Sanfilippo disease
FPO???
genua valgus
V-shaped configuration of the digits
hook-shaped appearance of L1vertebra
ovoid configuration of the vertebral bodies
rmment
'
9°)th
.
56. Acute inte
could
(NP) be the
all
exacerbated
by
.
followmg
EXCEPT
A- Surgery
3- Puberty
C. Pregnancy
138
D. premenstrual period
E. decreased carbohydrate intake
57. All the following drugs are unsafe in acute intermittent porphyria EXCEPT
A. nifedipine
B. diclofenac
C. cimetidine
D. valproic acid
E. carbamazepine
58. All the following are clinical features of acute intermittent porphyria EXCEPT
neuropathy
tachycardia
91.0.0???
abdominal pain
bladder dysfunction
cutaneous photosensitivity
61. All the following are features of congenital erythropoietic porphyria (CEP)EXCEPT
A. neuropathy
B. reddish urine
C. erythrodontia
D. photosensitivity
E. neonatal hyperbilirubinemia
139
porphyrins
D. erythrocyte
porphobilinogen
E. urinary
and
infants hypoglycemia
children, asWhole
isdefined 00dSilicose
63Inolder than
less
concentration
A. 45 mg/dL
B. 50 mg/dL
C. 55 mg/dL
D. 60 mg/dL
E. 65 mg/dL
long-term
well-defined
64.Theleast ofsevere
sequelae prolonged
hypoglycemia
is
A. cerebral palsy
B. personalityaffection
C. cognitive impairment
0. recurrent seizure activity
E. autonomic dysregulation
140
C. ketotic hypoglycemia
D. hereditary fructose intolerance
E. medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
meow
hypothyroidism
anorexia nervosa
nephrotic syndrome
72. All the following drugs are secondary causes of hypertrigiyceridemia EXCEPT
tegretol
estrogen
WP??? thiazides
Bblockers
anabolic steroids
141
LemIi-Opitz
73Smith (SLOS)
Syndrome byallthefollowing
ischaracterized EXCEPT
A. epispadias
B. cleft palate
C. microcephaly
D. retromicrognathia
E. mental retardation
laboratoryfindingsof medium-chain
74.Allthefollowingarerecognized acyl-CoA
dehydrogenase(MCAD)deficiency EXCEPT
A hypoglycemia
B. hypoketonuria
C. hypoketonemia
D. metabolic acidosis
E. elevated liver enzyme
75. A 3-year-old boy presented to emergency unit at 10:00 AM with history of difficult
arousal from sleep, lethargy, vomiting, and seizures; the mother stated that he had
slept without having his supper. Fasting blood sugar was 38mg/dL and urinary ketone
was low.
Of the following, the MOST likely diagnosis is
A. galactosemia
B. propionic acidemia
ketotic hypoglycemia
77. All the following are clinical abnormalities of Zellweger syndrome EXCEPT
A. cataract
B. micrognathia
C. high forehead
D. small fontanells
E. redundant skin fold of neck
142
impaired liver function
W90?
severely impaired hearing
chondrodysplasia punctata
pigmentary degeneration of the retina
79. All the following are characteristic features of infantile Refsum disease EXCEPT
A ataxic gait
B. hypertonia
C. hearingloss
D. impaired cognition
E. retinal degeneration
eye
ear
990?? skin
liver
heart
143
offattyacidoxidationdisorders
cluetothediagnosis
84.Thespecific maybethe
finding of hypoglycemiaand
hypoketonuria
metabolic acidosis
90???
.
elevated liver enzyme
elevated blood ammonia
prolonged prothrombln time
144
- Metaoolic Disoroers
ANSWERS
3
{HAHQALAARJI
1.(D). Many childhood conditions are caused by single-gene mutations that encode
specific proteins. These mutations can result in the alteration of primary protein
structure or the amount of protein synthesized. The function of a protein, whether it is
an enzyme, receptor, transport vehicle, membrane component, or structural element,
may be compromised or abolished.
2.(B). The earlier the appearance of clinical symptoms, the more severe is the disease.
3.(B). Replacement of the mutant gene with a normal one (gene therapy) is still in the
experimental phase.
4.(E). Elevation of blood ammonia is usually caused by defects of urea cycle enzymes.
Infants with elevated blood ammonia levels from urea cycle defects commonly have
normal serum pH and bicarbonate values; without measurement of blood ammonia,
they may remain undiagnosed and succumb to their disease.
5.(E).
6.(A). Phenylketonurea is the most likely diagnosis; these children have an unpleasant
odor of phenylacetic acid, which has been described as musty or mousey.
> Hawkinsinuria - ---Swimming pool
> Isovaleric acidemias Sweaty feet
> Trimethylaminuria Rotting fish
> - Boiled cabbage, rancid butte
Tyrosinemia
7.(D). Phenylketonurea is the most likely diagnosis; deficiency of the enzyme
phenylalanine hydroxylase (PAH) or of its cofactor tetrahydrobiopterin (8H4) causes
accumulation of phenylalanine in body fluids and in the brain.
8.(E). In infants with positive screening results, diagnosis should be confirmed by
quantitative measurement of plasma phenylalanine concentration.
9.(B). It is recommended that the blood for screening be obtained in the first 24-48 hr
of life after feeding protein to reduce the possibility of false-negative results, especially
in the milder forms of the condition.
10.(E). The mainstay of treatment of PKU is a lowphenylalanine diet. Most physicians
advocate phenylalaninerestricted diet'in patients with mild hyperphenylalaninemia
whose levels are persistently above 6 mg/ dL (360 umole/L). It is generally accepted
that infants with persistent (more than a few days) plasma levels of phenylalanine 26
mg/dL (360 umole/L) should be treated with a phenylalanine-restricted diet similar to
that for classic PKU.
11.(D). Tyrosinemia is the most likely diagnosis; these children have an unpleasant odor
which has been described as boiled cabbage, rancid butter.
145
12.(E).Tyrocinemia type 1 is a severe disease of the liver, kidney, and peripheral nerves
are caused by a deficiency of the enzyme fumarylacetoacetate hydrolase.
> acid B-glucosidase ----------------- Gaucher disease
> phenylalanine hydroxylase ------ Phenylketonurea
> acid sphingomyelinase ---- Niemann-pick disease
> fumarylacetoacetate hydrolase ------- Tyrosinemia
> Bhexosaminidases ----------- =-- Tay-Sachs disease
13.(D). Tyrocinemia type 1 is a severe disease of the liver, kidney, and peripheral nerves
are caused by a deficiency of the enzyme fumarylacetoacetate hydrolase. An acute
hepatic crisis commonly heralds the onset of the disease and is usually precipitated by
an intercurrent illness that produces a catabolic state. Episodes of acute peripheral
neuropathy occur in approximately 40% of affected children. Renal nvolvement is
manifested as a Fanconi-like syndrome.
14.(D). The presence of elevated levels of succinylacetone in serum and urine is
diagnostic for tyrosinemia type i. Increased levels of a-fetOprotein are present in the
cord blood of affected infants, indicating intrauterine liver damage. Plasma tyrosine
levels are usually elevated at diagnosis but this is a nonspecific finding and is
dependent on dietary intake. Plasma levels of other amino acids, particularly
methionine, may also be elevated in patients with liver damage. The urinary level of 5-
aminolevulinic acid is elevated because of inhibition of S-aminolevulinic hydratase by
succinylacetone.
15.(E). ln maple syrup urine disease, affected infants who are normal at birth develop
poor feeding and vomiting in the lst wk of life; lethargy and coma may ensue within a
few days. Physical examination reveals hypertonicity and muscular rigidity with severe
opisthotonos. Periods of hypertonicity may alternate with bouts of flaccidity
manifested as repetitive movements of the extremities (boxing and bicycling). Cerebral
edema may be present; convulsions occur in most infants, and hypoglycemia is
common.
16.(B). Treatment of the acute state of maple syrup urine disease is aimed at hydration
and rapid removal of the branched-chain amino acids and their metabolites from the
tissues and body fluids. Because renal clearance of these compounds is poor, hydration
alone may not produce a rapid improvement. Peritoneal dialysis or, preferably,
hemodialysis is the most effective mode of therapy in critically ill infants.
17.(D). Ocular manifestations of albinism include hypopigmentation of iris and retina,
foveal hypoplasia, reduced visual acuity, refractive errors, nystagmus, alternating
strabismus, and a red reflex.
18.(D). Homocystinuria; this is the most common inborn error of methionine
metabolism. Approximately 40% of affected patients respond to high doses of vitamin
B6 and usually have milder clinical manifestations than those who are unresponsive to
vitamin 86 therapy. These patients possess some residual enzyme activity.
In Homocystinuria,
19.(C). thromboembolic involvingboth largeandsmall
episodes
vessels,especiallythose of the brain, are common and may occur at any age. Optic
146
atrophy, paralysis, cor pulmonale, and severe hypertension (from renal infarcts) are
among the serious consequences of thromboembolism, which is caused by changes in
the vascular walls and increased platelet adhesiveness secondary to elevated
homocystine levels.
20.(D). Treatment with high doses of vitamin 36 (ZOO1,000 mg/24 hr) causes dramatic
improvement in most patients who are responsive to this therapy.
21.(D). Infants with homocystinuria are normal at birth. Clinical manifestations during
infancy are nonspecific and may include failure to thrive and developmental delay. The
diagnosis is usually made after 3 yr of age, when subluxation of the ocular lens (ectopia
lentis) occurs.
22.(D). Tay-Sachs disease results from the deficiency of Bhexosaminidase activity.
Clinical manifestations in infancy including loss of motor skills, increased startle
reaction, and macular pallor and retinal cherry-red spots.
23.(D). Both of Sandhoff disease and Tay-Sachs disease in infant characterize by a
neurologic features and retinal cherry-red spots. Infants with Sandhoff disease have
hepatosplenomegaly, cardiac involvement, and mild bony abnormalities.
24.(A). Gaucher disease is a multisystemic lipidosis characterized by hematologic
abnormalities, organomegaly, and skeletal involvement, the latter usually manifesting
as bone pain and pathologic fractures. It is one of the most common lysosomal storage
diseases, results from the deficient activity of the lysosomal hydrolase, acid B-
glucosidase, which is encoded by a gene located on chromosome 1q21q31. The
pathologic hallmark of Gaucher disease is the Gaucher cell in the reticuloendothelial
system, particularly in the bone marrow.
25.(C). Treatment of patients with Gaucher disease type 1 includes enzyme
replacement therapy. The efficacy of enzyme replacement therapy with mannose-
terminated recombinant human acid B-glucosidase has definitively been demonstrated.
Most symptoms (organomegaly, hematologic indices, bone pain) are reversed by
enzyme replacement therapy (60 IU/kg) administered by intravenous infusion every
other week and the bone involvement can be stabilized or improved.
26.(E). Niemann-pick disease (NPD), inherited as autosomal recessive traits, the clinical
manifestations and course of type A NPD is uniform and is characterized by a normal
appearance at birth. Hepatosplenomegaly, moderate lymphadenopathy, and
psychomotor retardation are evident by 6 mo of age, followed by neurodevelopmental
regression and death by 3 yr. With advancing age, the loss of motor function and the
deterioration of intellectual capabilities are progressively debilitating; and in later
stages, spasticity and rigidity are evident. Affected infants lose contact with their
environment. Type B disease is a non-neuronopathic form observed in children and
adults NPD types A and 8 result from the deficient activity of acid sphingomyelinase.
Type C disease is a neuronopathic form that results from defective cholesterol
transport.
27.(C). Niemann-pick disease type B patients do not have neurologic involvement and
have a normal IQ. Some patients with type 8 disease have cherry-red maculae or haloes
147
and subtle neurologic symptoms(peripheral neuropathy). In some type 8 patients,
decreased pulmonary diffusion caused by alveolar infiltration becomes evident in late
childhood or early adulthood and progresses with age. Prolonged neonatal jaundice
occurs in type C.
28.(A). Fabry disease, inherited as X-linked recessive.
29.(C). The progressive deposition of sphingomyelin in the central nervous system
results in the neurodegenerative course seen in type A, and in nonneural tissue in the
systemic disease manifestations of type 8, including progressive lung disease in some
patients.
30.(E). In type | GSD (von Gierke disease), despite marked hepatomegaly, the liver
transaminase levels are usually normal or only slightly elevated.
31.(E). ln type I GSD (von Gierke disease), easy bruising and epistaxis are common and
are associated with a prolonged bleeding time as a result of impaired platelet
aggregation and adhesion.
32.(C). Type I GSD (von Gierke disease) complicated by pancreatitis, renal failure,
hepatic adenoma, and pulmonary hypertension, while cardiomyopathy occure in type
II GSD (pompe disease).
33.(D). In patients with GSD lb, the loss of mucosal barrier function as a result of
inflammation, which is likely related to the disturbed neutrophil function, seems to be
the main cause of diarrhea.
34.(B). The diagnosis of type I GSD is suspected on the basis of clinical presentation and
the laboratory findings of hypoglycemia, lactic acidosis, hyperuricemia, and
hyperlipidemia. Neutropenia is noted in GSD lb patients, typically before 1 yr of age.
Administration of glucagon or epinephrine results in little or no rise in blood glucose
level, but the lactate level rises significantly. Before the glucose- 6-phosphatase and
glucose-G-phosphate translocase genes were cloned, a definitive diagnosis required a
liver biopsy. Gene-based mutation analysis provides a noninvasive way to diagnose
most patients with types la and lb disease.
35.(C). Sucrose (table sugar, cane sugar, other ingredients), fructose (fruit, juice, high
fructose corn syrup), lactose (dairy foods), and sorbitol should be avoided or limited.
36.05). The confirmatory step for a diagnosis of Pompe disease is enzyme assay
demonstrating deficient acid a-glucosidase. The enzyme assay is usually done in dried
blood spots, leukocytes, blood mononuclear cells, muscle, and cultured skin fibroblasts.
37.(C). Specific enzyme replacement therapy (ERT) with recombinant human acid a-
glucosidase is available for treatment of Pompe disease and is capable of preventing
deterioration or reversing abnormal cardiac and skeletal muscle functions. A high-
protein diet and exercise therapy may also be beneficial. Nocturnal ventilatory support,
when indicated, should be used. It has been shown to improve the quality of life and is
particularly beneficial during a period of respiratory decompensation.
38.(C). ln galactosemia, without the transferase enzyme, the infant is unable to
metabolize galactose-l-phosphate, the accumulation of which results in injury to
kidney, liver, and brain.
148
39.(E). In galactosemia when the diagnosis is not made at birth, damage to the liver
(cirrhosis) and brain (intellectual disability) becomes increasingly severe and
irreversible.
40.(A). Patients with galactosemia are at increased risk for Escherichia coli neonatal
sepsis; the onset of sepsis often precedes the diagnosis of galactosemia.
41.(E). Patients with hereditary fructose intolerance HFl are asymptomatic until
fructose or sucrose (table sugar) is ingested (usually from fruit, fruitjuice, or sweetened
cereal). Symptoms may occur early in life, soon after birth if foods or formulas
containing these sugars are introduced into the diet.
42.(E). In hereditary fructose intolerance, intellectual development is usually
unimpaired.
43.(A). In hereditary fructose intolerance, treatment consists of the complete
elimination of all sources of sucrose, fructose, and sorbitol from the diet. It may be
difficult because these sugars are widely used additives, found even in most medicinal
preparations.
44.(A). Hurler syndrome; Radiographs show a characteristic skeletal dysplasia known as
dysostosis multiplex. The earliest radiographic signs are thick ribs and ovoid vertebral
bodies. Skeletal abnormalities include enlarged, coarsely trabeculated diaphyses of the
long bones with irregular metaphyses and epiphyses. With progression of the disease
macrocephaly develops with thickened calvarium, premature closure of lambdoid and
sagittal sutures, shallow orbits, enlarged J-shaped sella, and abnormal spacing of teeth
with dentigerous cyst.
4S.(D). Enzyme replacement using recombinant a-L-iduronidase has been approved for
patients with MPS-l. It reduces organomegaly and ameliorates rate of growth, joint
mobility, reduces the number of episodes of sleep apnea and urinary GAG excretion.
The enzyme does not cross the bloodbrain barrier and does not prevent deterioration
of cognition and other neurologic functions.
46.(B). Scheie is a comparatively mild disorder characterized by joint stiffness, aortic
valve disease, corneal clouding, and mild dysostosis multiplex. Patients with Scheie
disease have normal intelligence and stature but have significant joint and ocular
involvement.
47.(B). Patients with severe MPS-II have features similar to those of Hurler disease
except for the lack of corneal clouding and the somewhat slower progression of
somatic and central nervous system (CNS)deterioration.
48.(C). Hunter disease inherited as X- linked recessive.
4S.(D). Morquio disease is characterized by short-trunk dwarfism, fine corneal deposits,
a skeletal dysplasiathat is distinct from other mucopolysaccharidoses,and preservation
of intelligence.
50.(B).
51.(E). Patients with Sanfilippo disease are characterized by slowly progressive, severe
CNSinvolvement with mild somatic disease. Such disproportionate involvement of the
CNS is unique to MPs-III.
149
52.(E). Bulletshaped phalanges in addition to metacarpals and phalanges are
abnormally short, wide and deformed with proximal pointing of the metacarpals are
feature of Hurler disease.
5340). In Morquio disease, instability of the odontoid process and ligamentous laxity is
regularly present and can result in life~threatening atlantoaxial instability and
dislocation.
54.(D). Hurler disease, anterosuperior hypoplasia of l.-1 resulting in hook-shaped
appearance.Sanfilippo disease, immature, ovoid configuration of the vertebral
bodies.Morquio disease, genua valgus and kyphosis. Scheie disease, the carpal bones
are small leading to V-shaped configuration of the digits.
55(8). Any individual who is suspected of a mucopolysaccharidoses disorder based on
clinical features, radiographic results, or urinary GAG screening tests should have a
definitive diagnosis established by enzyme assay. Serum, leukocytes, or cultured
fibroblasts are used as the tissue source for measuring lysosomal enzymes.
56.(C). Surprisingly, pregnancy is usually well tolerated, suggesting that beneficial
metabolic changes may ameliorate the effects of high levels of progesterone.
57.(C).
58.(E). Cutaneous photosensitivity is a feature of Porphyria cutanea tarda.
59.(D). The common manifestations include mental symptoms; pain in the extremities,
head, neck, or chest; muscle weakness; and sensory loss. Because all these
manifestations are neurologic rather than inflammatory, there is little or no abdominal
tenderness, fever, or leukocytosis.
60(8). Hyponatremia is common during acute attacks. Inappropriate antidiuretic
hormone secretion is often the most likely mechanism, but salt depletion from excess
renal sodium loss, gastrointestinal loss, and poor intake have been suggested as causes
of hyponatremia in some patients. Other electrolyte abnormalities may include
hypomagnesemiaand hypercalcemia.
61.(A). Neuropathic symptoms are absent, and there is no sensitivity to drugs,
hormones, and carbohydrate restriction.
62.(E).
> Porphyria cutanea tarda ------- - Plasma (or urine) porphyrins.
> Erythropoietic protoporphyria --~ Erythrocyte (or plasma) porphyrins.
63.(C).in older infants and children, a whole blood glucose concentration of <55 mg/dL
(1015% higher for serum or plasma) represents hypoglycemia, because counter
regulatory mechanismsare activated at these glucose concentrations.
64(8). Subtle effects on personality are also possible but have not been clearly defined.
65.(D). Thyroid hormone is not a counter regulatory hormone in response to
hypoglycemia.
66.(C).Children with ketotic hypoglycemia have plasma alanine concentrations that are
markedly reduced in the basal state after an overnight fast and decline even further
with prolonged fasting. Alanine, produced in muscle, is a major gluconeogenic
precursor.
150
67.(C). During acute episodes of medium-chain acyl-CoA dehydrogenase (MCAD)
deficiency, hypoglycemia is usually present; plasma and urinary ketone concentrations
are inappropriately low (hypoketotic hypoglycemia).
68(8). During intercurrent illnesses, parents should be taught to test the childs urine
for the presence of ketones, the appearance of which precedes hypoglycemia by
several hours. In the presence of ketonuria, liquids of high carbohydrate content should
be offered to the child.
69.(D). The most specific and important laboratory finding is the demonstration of
abnormally high levels of VLCFA in plasma, red blood cells, or cultured skin fibroblasts.
The test should be performed in a laboratory that has experience with this specialized
procedure. Positive results are obtained in all male patients with ALD and in
approximately 85% of female carriers of ALD.
70.(D). In 80% of patients, the lesions are symmetric and involve the periventricular
white matter in the posterior parietal and occipital lobes. Approximately 50% show a
garland of accumulated contrast material adjacent and anterior to the posterior
hypodense lesions. In 12% of patients, the initial lesions are frontal.
71.(A). Obesity is secondary cause of hypertriglyceridemia.
72.(A). Tegretol is secondary causes of hypercholesterolemia.
73.(A). Genital anomalies; hypospadias, cryptorchidism, and sexual ambiguity (<50%).
74.(D). During acute episodes of MCAD deficiency, hypoglycemia is usually present.
Plasma and urinary ketone concentrations are inappropriately low (hypoketotic
hypoglycemia). Because of the hypoketonemia, there is little or no metabolic acidosis,
which is expected to be present in many children with hypoglycemia.
75.(E). MCAD; Previously undiagnosed affected patients usually present in the first
3 mo-S yr of life with episodes of acute illness triggered by prolonged fasting (longer
than 12-16 hr). During acute episodes of MCAD deficiency, hypoglycemia is usually
present. Plasma and urinary ketone concentrations are inappropriately low
(hypoketotic hypoglycemia).
76.(B). The most common presentation of primary carnitine deficiency is progressive
cardiomyopathy with or without skeletal muscle weakness beginning at 1-4 yr of age.
77.(D). Craniofacial dysmorphisms are; high forehead, flat occiput, large fontanelle(s),
wide sutures, shallow orbital ridges, low/broad nasal bridge, epicanthus, high arched
palate, external ear deformity, and micrognathia.
78.(D). Chondrodysplasia punctata and renal cysts are absent.
79(8). Early hypotonia and hepatomegaly with impaired function are common.
80(5).
> Neonatal adrenoleukodystrophy --- Phytanic acid (increased).
> Infantile Refsum disease ---- Pristanic acid (increased), Bile acid (Increased), and
Plasmalogen level (decreased).
81.(D). In progeria; normally functioning systems are; Liver, kidney, thyroid, immune,
gastrointestinal, and neurologic systems (other than stroke-related) remain intact.
Intellect is normal for age.
151
82.(B). In the childhood cerebral form of ALD, symptoms are first noted most
commonly between the ages of 4 and 8 yr. The most common initial manifestations are
hyperactivity. which is often mistaken for an attention deficit disorder, and worsening
school performance in a child who had previously been a good student.
83.10). The most common presentation is an acute episode of life-threatening coma
and hypoglycemia induced by a period of fasting because of defective hepatic
ketogenesis.
84.(A). Fatty acid oxidation disorders are easily overlooked because the only
specific clue to the diagnosis may be the finding of inappropriately low
concentrations of urinary ketones in an infant who has hypoglycemia.
851(2). Familial combined hyperlipidemia; an autosomal dominant condition
characterized by moderate elevation in plasma LDL cholesterol and triglycerides, and
reduced plasma HDL cholesterol.
152
- TheFetusandtheNeonatal
Infant
QUESTIONS
ALMUSAWI
HAIR
éu
1. Majorcauseof neonatalmortality in full-term newbornis
A. respiratorydistresssyndrome
necrotizing enterocolitis
.097
bronchopulmonary dysplasia (BPD)
D. congenital anomalies
E. intraventricular hemorrhage
2. Generalized edema of the newborn may occur in the following conditions EXCEPT
A. prematurity
B. nonimmune hydrops
C. Turner syndrome
D. congenital nephrosis
E. Hurler syndrome
andwormianbone
3. Softareasin the occipitalregionsuggestthe irregularcalcification
formation usually associated with the following conditions EXCEPT
osteogenesis imperfecta
craniosynostosis
W909"?
cleidocranial dysostosis
cretinism
Down syndrome
with a largeanteriorfontanelEXCEPT
4.Thefollowingdisordersare associated
A. congenital rubella syndrome
B. hypophosphatasia
C. Apert syndrome
D. Russell-Silversyndrome
E. vitamin A deficiency
153
arte
umbilical
6.Asingle
A. meningomyelocele
anomaly
B.occultrenal
C. omphalocele
D. gastroschisis
E. omphalitis
7. Thefollowin
drugs
A. psychotropic
B. amphetamines
C. bromocriptine
D. chloramphenicol
E. methimazole
with
associated
is
which
condition is
polyhydramnios
the
8.Of the
following,
A renal
agenesissyndrome)
(Potter
8. Prune-bellysyndrome
C. pulmonaryhypoplasia
D. intestinal
pseudo-obstruction
E. hernia
diaphragmatic
serum
maternal
9.Low u-fetoprotein with
isassociated
(MSAFP)
A. openneuraltubedefects
8. trisomy 21
C. gastroschisis
D. omphalocele
E. congenital nephrosis
POP?premature
narcotic
areseen
in
rupture of the fetal membranes
addiction
maternal hypertensive
'
ths and
and renal vascular disease
a '
ssoctated
are
'
a roxim
Pp 1
V0Of
EXCEPT ately
11.Trueumbilicalcordknots
. the
With conditions
followmg
short cord
small fetal size
990??
p("Ythramnios
monoamniotic
fetal demise
twinning
154
multiplegestation
12,Regarding thefollowing
pregnancies, aretrueEXCEPT
A. thereportedinCIdence
of spontaneous islowest
twinning intheAsian
races
to occurin 1 in862pregnancies
5, tripletsareestimated intheUSA
of
c. theincidence monozygotic twins isunaffectedbyracialorfamilialfactors
0. theoverall incidence
of multifetal
gestationis unchanged
are morefrequentbeyondthe 2ndpregnancy
pregnancies
5. polyovular
14. One of the following drugs may cause pyloric stenosisif administeredto a
premature infant
A. intravenousvitaminE
B. indomethacin
C. entericgentamicin
D. prostaglandins
E. dexamethasone
17-Sudden
onset
ofhypotension infant
(VLBW)
inaverylowbirthweight suggests
A- pneumothorax
3. necrotizing
enterocolitis
155
sepsis
C. bacterial
D. myocarditis
E. hypoglycemia
or
room
delivery
the
in shortly bedue
thereafter
may to
beginning
Seizures
18.
encephalopathy
A. hypoxic-ischemic .Into fetus
the
ofmaternal
injection
B.unintentional anesthetic
local
hemorrhage
C. intracranial
D. cerebralanomaly
E. hypoglycemia
birth
severe infants motor
have
may characterized
automatisms by
19.After asphyxia,
movements
oforal-buccal-lingual
A. abscence
discharges
electroencephalographic
B. time-synchronized
cortical
C. significant activity
epileptic
D. goodresponse therapy
to anticonvulsant
E. a poor prognosis
intheneonatal
20.Vomiting dueto
periodisusually
A. pyloric stenosis
B. milk allergy
C. overfeeding
D. stress ulcer
E. an inborn error of metabolism
156
exposure
23. Earlypostnatal to dexamethasone,
withinthe lst wk of life,in VLBw
is
infants, with
associated the EXCEPT
following
A. metabolicderangements
8. poorgrowth
C. increasedriskfor sepsis
D. increasedriskof spontaneousbowel perforation
E. cerebral palsy
09?;
apnea is a common problem in preterm infants
in term infants, apnea is always worrisome
obstructive apnea is characterized by absence of airflow but persistent chest
wall motion
D. seriousapnea is defined as cessation of breathing for longer than 20 sec
l'" bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often nodal
25. The risk for development of respiratory distress syndrome RDS increases with the
following conditions EXCEPT
prolonged rupture of membranes
maternal diabetes
26. The following are true regarding respiratory distress syndrome (RDS) EXCEPT
A. signsof ROSusually appear within minutes of birth
B. in mostcases,the symptomsand signsreach a peak within 7days
C. apnea and irregular respirations are ominous signs
D. improvement is often heralded by spontaneous diuresis
E. mixed respiratory-metabolic acidosismay be seen
28~Administration to women
corticosteroids
of antenatal 24and34wkof
between
gestationsignificantlyreduces the following EXCEPT
A. incidence
andmortalityof R05
157
postnatalgrowth
the overall neonatal mortality
wen???
circulatory insufficiency
hypothermia . .
of oxygenandcarbondiox1de
pulmonaryexchange
inadequate
electrolytes disturbance
31. Prophylactic and rescue administrations of synthetic and natural surfactants have
the following advantages EXCEPT
A. reduces adverse outcomes
8. reduces neonatal mortality
C. decreses the risk for pneumothorax
D. decreses the risk for pulmonary interstitial emphysema
E. reduces bronco~pulmonary dysplasia BPD rates
158
factorsfor the development
34,Contributary of broncho-
pulmonarydysplasia(BPD)
thefollowingEXCEPT
lnclude
immaturity
dehydrationduringthe lst daysof life
F905?chorioamnionitis
symptomatic PDA
malnutrition
VIP???
improve diaphragmatic contractility
increase pulmonary vascular resistance PVR
diuretic effects
36. One of the following is FALSEin the chest radiograph of transient tachypnea of the
newborn (Ti'N)
A. prominent pulmonary vascular markings
fluid in the intralobar fissures
[9059
overaeration
flat diaphragms
diffuse reticulogranular pattern
wpnp
3-5% die
overdistention of the chest may be prominent
usuallyoccursin preterm or near-terminfants
38.Thefollowing forpersistent
factors
arepredisposing pulmonary ofthe
hypertension
newborn (PPHN) EXCEPT
A. anemia
8. meconiumaspirationsyndrome
C. early-onset sepsis
D. hypoglycemia
E. birthasphyxia
39-Associated
anomalies
havebeen
reported diaphragmatic
inupto30%0fcongenital
hernia EXCEPT
theseincludethefollowing
cases;
A. pulmonaryhypoplasia
159
CNS lesions
atresia
£11.00!hernia(CDH)canbediagnosed
esophageal
omphaiocele
lesions
cardiovascular
onprenatal
diaphragmatic
40. Congenital in>50%ofcases.
16
(between
ultrasonography 24wkofgestation)
and
include
may
onultrasonography EXCEPT
thefollowing
Findings
A. chest mass
8. mediastinalshift
C. gastricbubble
D. olighydramnios
E. a liver in the thoraciccavity
41.Apoor sign
prognosticofcongenital hernia
diaphragmatic is
(CDH)
grunting
use of accessorymuscles
W909?earlyrespiratory
cyanosis
scaphoid abdomen
within6 hr of life
distress,
42. Eventration of the diaphragm may be associated with the following EXCEPT
pulmonary hypopiasia
pulmonary sequestration
9.00.?
congenital heart disease
chromosomal
recurrent
trisomies
infections
- - ' '
43. All the following are true. regarding pathology of neonataln ente
ecrotlzmgrocolitis
EXCEPT
(NEC)
A. in fatal cases,gangrenemay extendfrom the stomachto
the rectum
B the greatestriskfactorfor NECis
prematurity
C. in most situations,a pathogen is identified
D. NEC muchlesscommonin infantsfed
is humanmilk
E. NECin term infantsisoftena
"secondarydisease
44. Absolute indication for sur ' -
.,
-'
88W In neonatal
g me
enterocolitis
(NEC) |ude
necrotizin
posmve result
ofabdominal
Paracentesis
failureof medicalmanagement
160
45.Thefollowingfactors
are associated
withdecreased
riskof significant
jaundice
EXCEPT
A gestational age 241 wk
B. male gender
C. exclusive bottlefeeding
D. black race
E. discharge from hospital after 72 hr
47. A search to determine the cause of jaundice should be made in all the following
conditions EXCEPT
A if it appears in the first 24-36 hr of life
B. if serum bilirubin is rising at a rate faster than 5 mg/dL/24 hr
C. if serum bilirubin is >12 mg/dL in a fullterm infant
D. ifjaundice persists after 10-14 days of life
E. if direct bilirubin fraction is >1 mg/dL at any time
48. Regarding breast milk jaundice, the following are true EXCEPT
A. it develops in an estimated 2% of breast-fed term infants
B. maximalunconjugatedbilirubin concentrationsas high as 10-30mg/dL reached
during the 2nd-3rd week
C. jaundice may persist for 310 wk
D. phototherapy may be of benefit
E. kernicterus never occur
50.Early
signsof hydropsinclude
ultrasonographic
A. double-bowel wall sign (bowel edema)
B.
lehydramnios
161
C. ascites
D. pleuraleffusions
E. scalpedema
isFAISE
52.Oneofthefollowing disease
inhemolytic caused
ofthenewborn byblood
group A and B incompatibility
A. occursin 20-25%of pregnancies
ABOincompatibility
B. A1 is more antigenic than A2
C. natural antibodiesagainstA and 8 factors are usually lgM antibodies that do
not cross the placenta
D. isoimmune hemolytic disease may be found in first-born infants irrespective of
infant blood group
most cases are mild
POP? maternalantibodytiterscanhelppredictthe
severe anemia is frequent
stillbirth/hydropsisfrequent
severityof fetal disease
central values
the incidenceof neonatal '
Polycythemial5 8% in small for
infants gestational age (56A)
neonatalRBCshave
decreased
tostasis
inthe
microcirculation and
d8formability which
filterability,
predispose
morefrequentin breast-fed
than informula-fed
infants
bleedingtime is normal
POP
oralvitaminK is as effectiveas intramuscular
treatment of the disease
routein the preventiOn
and
E. time(PT),andpartialthromboplastin
prothrombin time(PTT)
areprolonged
57. The normal cord at term is 55 cm long. Abnormally short cordsare associatedwith
the following EXCEPT
A. fetal hypotonia
FPO?
uterine constraint
oligohydramnios
increased risk for complications of labor and delivery for both mother and
infant
59. Generalizededema may be seen in the neonatal period with the following
conditions EXCEPT
congenital nephrosis
Turner syndrome
W905?
Hurler syndrome
hydrops fetalis
offspring of diabetic mothers
POP?
preeclampsia
pyelonephritis
Preterm labor
163
E. chronichypertension
mothers.
ofdiabetic
61.inlnfants aretrueEXCEPT
thefollowing
inabout finfants mothers
ofdiabetic
A. hypoglycemia
develops 25-50%.0
8. increased
weight andallinfant
oftheplacenta organs
C.heart occurs
failure ofdiabetic
ofinfants
in5-10% mothers
. in
. fantsof
ofcongenital
D. theincidence is
anomalies d in
threefol
increased
diabetic
mothers ' anoma
E Iumbosacral common
isoneofmost congenital l'ies
agenesis
62.Thefollowing aretrueEXCEPT
definitions '.
A. a syndromeisdefinedasa patternof multiple thatarerelatedby
abnormalities
andresultfroma commondefinedetiology
pathophysiology
B. dysplasiasequenceis a poororganizationof cellsinto tissuesor organs
C. an association refers to a nonrandom collection of malformations in which
there is an unclear relationship among the malformations
D. disruption sequence is a mechanical (uterine) forces that alter structure of
intrinsically normal tissue
E. malformationsequenceis a single,localtissuemorphogenesisabnormality that
producesa chain of subsequent defects
164
a, variceiia
55.Agents cause
thatcommonly nosocomialneonatal
Infection
are
GBS
groupBstreptococci
coagulasemegativestaphylococci
WP???proteus
gonococci
chlamydiae
68. in a cohort of 6,215 VLBW infants in the NlCHD Neonatal ResearchNetwork, gram~
positiveagents were associated with which percent of cases of late-onset sepsis
A 90%
B. 70%
C. 5096
D. 30%
E. 10%
7o. intracranial
calcificationmay be a feature of one of the followingtransplacental
infections
cytomegalovirus
herpes simplex virus
POP? varicella-zoster
syphilis
rubella
71'Mb hVpoplasia
virus
infections
transplacental
ofthefollowing
maybeafeature
A wtomegalovlrus
B. herpessimplexvirus
185
virus
C. varicella-zoster
D. HIV
E. rubella
72.Common manifestations
tract
respiratory infections
bacterial
ofneonatal is
ethmoiditis
otitis media
W909?mastoiditis
retropharyngealcellulitis
empyema
manifestations
skinandsofttissue
73. Common infections
bacterial
ofneonatal are
facial cellulitis
scalp abscess
F1909?
fasciitis
breast abscess
omphalitis
74. One of the following is not included in the definition of the systemic inflammatory
response (SIRS) in neonates and pediatric patients
temperature instability
abnormal white blood cell (WBC) count
FPDP?respiratory dysfunction
cardiac dysfunction
perfusion abnormalities
WP???Escherichia
Pseudomonas
coli
Candida albicans
76. Persistence
of the umbilical
cordbeyondwhichtime shouldpromptconsideration
of an underlying abnormality
A. 10 days
8. 20 days
C. 30 days
D. 40 days
E. 50 days
166
77.TheMOSTimportantrisk factor for necrotizingenterocolitis(NEC)in preterm
infantsis
A. delivery by cesarean section
breast feeding
apgar score
choose?pseudomembranous enterocolitis
neonatal
meconium
ulcerative
ileus
colitis
79. The following factors suggest hemolytic disease as a cause of jaundice in the
newborn EXCEPT
bilirubin rise of >0.5 mg/dL/h
reticulocytosis >5% at birth
80. W hich common sugar does the clinitest screen not detect?
A. glucose
8. fructose
C. galactose
D. sucrose
E lactose
187
D. fourth day of life
E. fifth day of life
160
m TheFetusandtheNeonatal
infant
ANSWERS
ALMUSAWI
zUHAIR
140). Severe immaturity, respiratory distress syndrome, intraventrlcular hemorrhage,
infection,necrotizing enterocolitis, and bronchopulmonary dysplasia (BPD) are major
causesof mortality in preterms.
2.(C). Localizededema suggests a congenital malformation of the lymphatic system;
whenconfinedto one or more extremities of a female infant; it may be the initial sign
of Turner syndrome.
1(8). Persistently small fontanels suggest microcephaly, craniosynostosis,and
congenitalhyperthyroidism.
44E). Vitamin D deficiency rickets is associated with a large anterior fontanel.
5.(C). A cornea >1cm in diameter in a term infant (with photophobia and tearing)
suggestscongenital glaucoma and requires prompt ophthalmologic consultation.
6.(B).A single umbilical artery increases the risk for an occult renal anomaly.
7.(A). Amphetamines, bromocriptine, chloramphenicol, and methimazole are
contraindicated in breast-feeding mother.
8.(E). Renal agenesis (Potter syndrome), Prune-belly syndrome, pulmonaryhypoplasia,
and intestinal pseudo-obstruction are associated with oiigohydramnios.
9.(B).Second-trimesterscreening (15-18 wk) of maternal serum a-fetoprotein (MSAFP)
values is used to screen for open neural tube defects. About 90% of affected
pregnanciescan be detected by an elevated MSAFP value. Gastroschisis,omphalocele.
congenitalnephrosis, twins, and other abnormal conditions can also be identified. Low
MSAFPis associated with incorrect gestational age estimates, trisomy 18 or 21, and
intrauterine growth restriction.
10.(A). Earlier lung maturation may occur in the presence of severe premature
separation of the placenta, premature rupture of the fetal membranes, narcotic
addiction, or maternal hypertensive and renal vascular disease. A delay in pulmonary
maturation may be associated with hydrops fetalis or maternal diabetes without
vascular disease.
11.(A). Short cords and noncoiled cords occur with chromosome abnormalities and
omphalocele.
12.(D). Although the incidence of spontaneous multifetal gestation has been stable
over the years, the overall incidence of multifetal gestation is increasing as a result of
treatment of infertility with ovarian stimulants (ciomiphene, gonadotropins) and in
vitro fertilization. Twins account for about 2.5% of births but about 20% of very low
birthweight(VLBW)infants.
13.(B). Respiratory distress syndrome (hyaiine membrane disease) and apnea are
commonneonatal respiratory problem associated with premature infants.
169
14.(D). Prostaglandins and erythromycin may cause pyloric stenosis if administered to a
premature infant.
15.(C).Antenatal exposure to magnesium sulfate may have neuroprotective effects and
may reduce the incidence of cerebral palsy in high-risk neonates.
16(8). Large for gestational age LGA infants, regardless of their gestational age, have a
higher incidence of birth injuries, such as cervical and brachial plexus injuries, phrenic
nerve damage with paralysis of the diaphragm, fractured clavicles, cephalohematomas,
subdural hematomas, and ecchymoses of the head and face. LGA infants are also at
increased risk for hypoglycemia and polycythemia. The incidence of congenital
anomalies, particularly congenital heart disease, is also higher in LGA infants than in
term infants of normal weight. Intellectual and developmental retardation is
statistically more common in high birthweight term and preterm infants than in babies
of appropriate weight for gestational age
17.(A). Sudden onset of hypotension in a very low birthweight (VLBW) infant suggests
pneumothorax, intraventricular hemorrhage, or subcapsular hepatic hematoma.
18.lB). Seizures beginning in the delivery room or shortly thereafter may be due to the
unintentional injection of maternal local anesthetic into the fetus. Convulsions may
also result from hyponatremia and water intoxication in the infant after the
administration of large amounts of hypotonic fluid to the mother shortly before and
during delivery.
19.(E). After severe birth asphyxia, infants may have motor automatisms characterized
by oral-buccal-lingual movements, rotary limb activities (rowing, pedaling, and
swimming), tonic posturing, or myoclonus. These motor activities are not usually
accompanied by time-synchronized electroencephalographic discharges, may not
signify cortical epileptic activity, respond poorly to anticonvulsant therapy, and are
associated with a poor prognosis. Such automatisms may represent cortical depression
that produces a brainstem release phenomenon or subcortical seizures.
20.(C). Vomiting during the lst day of life suggests obstruction in the upper digestive
tract or increased intracranial pressure. Roentgenographic studies are indicated when
obstruction is suspected. Vomiting may also be a nonspecific symptom of an illness
such as septicemia. It is a common manifestation of overfeeding, inexperienced feeding
technique, or normal reflux and is rarely due to pyloric stenosis, milk allergy, duodenal
ulcer, stress ulcer, an inborn error of metabolism (hyperammonemia, metabolic
acidosis),or adrenal insufficiency.
21(8). Cephalohematomais a subperiosteal hemorrhage, hence always limited to the
surface of one cranial bone, while caput succedaneum is a diffuse, sometimes
ecchymotic, edematous swelling of the soft tissues of the scalp involving the area
presenting during vertex delivery. It may extend across the midline and across suture
lines.
22.(B).Ultrasonographyis the preferred imaging technique for screening becauseit is
noninvasive,portable, reproducible, and sensitive and specific for detection of IVH.
170
23.(E).Infants exposed to postnatal steroids after the lst wk of life have an increased
risk of cerebral palsy and developmental delay.
24.(E).Bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often sinus, but on occasion it can be nodal. Vagal responses and, rarely, heart block
are causes of bradycardia without apnea.
25.(A).The risk of RDSis reduced in pregnancies with chronic or pregnancy-associated
hypertension, maternal heroin use, prolonged rupture of membranes, and antenatal
corticosteroid prophylaxis.
26(8). in most cases, the symptoms and signs reach a peak within 3 days, after which
improvement is gradual.
27.(C). in the differential
diagnosis, early-onset sepsis may be indistinguishable from
RDS.In pneumonia manifested at birth, the chest roentgenogram may be identical to
that for RDS. Maternal group B streptococcal colonization, identification of organisms
on gram staining of gastric or tracheal aspirates or a buffy coat smear, and/or the
presence of marked neutropenia may suggest the diagnosis of early-onset sepsis.
Cyanoticheart disease (total anomalous pulmonary venous return) can also mimic RDS
both clinically and radiographically. Echocardiography with color-flow imaging should
be performed in infants who show no response to surfactant replacement, to rule out
cyanotic congenital heart disease as well as ascertain patency of the ductus arteriosus
and assesspulmonary vascular resistance (PVR).
Z8.(B). Postnatal growth is not adversely affected. Antenatal steroids do not increase
the risk of maternal death, chorioamnionitis, or puerperal sepsis.
29.(D). The basic defect requiring treatment in RDS is inadequate pulmonary exchange
of oxygen and carbon dioxide; metabolic acidosis and circulatory insufficiency are
secondary manifestations.
30.(B). Warm humidified oxygen should be provided at a concentration initially
sufficient to keep arterial oxygen pressure between 50 and 70 mm Hg (91-95%
saturation) in order to maintain normal tissue oxygenation while minimizing the risk of
oxygen toxicity. lf oxygen saturation cannot be kept >90% at inspired oxygen
concentrations of 40-70% or greater, applying CPAP at a pressure of 5-10 cm H20 via
nasal prongs is indicated and usually produces a rapid improvement in oxygenation.
CPAP reduces collapse of surfactant~deficient alveoli and improves both FRC and
ventilation-perfusion matching.
31.(E).The lack of reduction in BPD rates following surfactant replacement is probably,
in part, due to the survival of infants with severe RDS who would have died without
surfactant administration.
32.(E). Inhaled nitric oxide (iNO) decreases the need for extracorporeal membrane
oxygenation (ECMO) in term and near-term infants with hypoxic respiratory failure or
persistent pulmonary hypertension of the neonate.
33.(E). Alkali therapy may result in skin slough from infiltration, increased serum
osmolarity, hypernatremia, hypocalcemia, hypokalemia, and liver injury when
171
concentrated solutions are administered rapidly through an umbilical vein catheter
Wedged in the liver.
3MB). Overhydration during the lst days of life may also contribute to the
development of BPD. Vitamin A supplementation (5,000 lU intramuscularly 3 times/wk
for 4 wk) in VLBW infants reduces the risk of BPD.
35.(D). Methylxanthines may also decrease PVR and increase lung compliance in infants
with BPD, probably through direct smooth muscle relaxation.
36.(E). The distinctive features of transient tachypnea are rapid recovery of the infant
and the absence of radiographic findings for RDS (hypoaeration, diffuse
reticulogranular pattern, air bronchograms) and other lung disorders.
37.(E). Meconiumstained amniotic fluid is found in 10-15% of births and usually occurs
in term or post-term infants.
38.(A). Persistent pulmonary hypertension of the newborn (PPHN) occurs in term and
post-term infants. Predisposing factors include birth asphyxia, MAS, early-onset sepsis,
RDS, hypoglycemia, polycythemia, maternal use of nonsteroidal anti-inflammatory
drugs with in utero constriction of the ductus arteriosus, maternal late trimester use of
selective serotonin reuptake inhibitors, and pulmonary hypoplasia due to
diaphragmatic hernia, amniotic fluid leak, oligohydramnios, or pleural effusions. PPHN
is often idiopathic.
39.(A). Pulmonary hypoplasia and malrotation of the intestine are part of the lesion,
not associated anomalies.
40.(D). Polyhydramnios
41.(C). Early respiratory distress, within 6 hr of life, is thought to be a poor prognostic
Sign.
42.(A). Congenital eventration may affect lung development, but it has not been
associated with pulmonary hypoplasia.
43.(C). Clustering of cases suggests a primary role for an infectious agent. Various
bacterial and viral agents, including Escherichia coli, Klebsiella, Clostridium perfringens,
Staphylococcus epidermidis, astrovirus, norovirus, and rotavirus, have been recovered
from cultures. Nonetheless, in most situations, no pathogen is identified.
44.(A). A surgeon should be consulted early in the course of treatment. Indications for
surgery include evidence of perforation on abdominal roentgenograms
(pneumoperitoneum) or positive result of abdominal paracentesis (stool or organism
on Gram stain preparation from peritoneal fluid). Failure of medical management, a
single fixed bowel loop on radiographs, abdominal wall erythema, and a palpable mass
are relative indications for exploratory laparotomy.
45(8). Male gender is a minor risk factor.
46.(B). laundice that first appears on the 2nd or 3rd day is usually physiologic but may
represent icterus(Crigler-Najjar
a moresevereform.Familialnonhemolytic syndrome)
and early-onset breast-feedingjaundice are seen initially on the 2nd or 3rd day.
47.(E).Direct bilirubin fraction is >2 mg/dL at any time.
172
48(E). Although uncommon, kernicterus can occur in patients with breast milk
jaundice.
49(8). Dark skin does not reduce the efficacy of photothera py.
50(A). Early ultrasonographic signs of hydrops include organomegaly (liver, spleen,
heart), the double-bowel wall sign (bowel edema), and placental thickening.
Progression to polyhydramnios, ascites, pleural or pericardial effusions, and skin or
scalp edema may then follow.
51.(E). The white blood cell count is usually normal but may be elevated.
52(0). Although antibodies against A and B factors occur without previous
immunization (natural" antibodies), they are usually lgM antibodies that do not cross
the placenta. However, lgG antibodies to A antigen may be present and these do cross
the placenta, so A-O isoimmune hemolytic disease may be found in first-born infants.
53(8). 5% of first-born infants may be affected.
54(8). Peripheral (heelstick) Hct values are higher than central values, whereas Coulter
counter results are lower than Hct values determined by microcentrifugation.
55(0). Although oral vitamin K (birth, discharge, 3-4 wk: 12 mg) has been suggested as
an alternative, oral vitamin K is less effective in preventing the late onset of bleeding
due to vitamin K deficiency and thus cannot be recommended for routine therapy. The
intramuscular route remains the method of choice.
56(8). Apt devised the following test for differentiation of fetal from maternal blood:
(1) Rinse a blood-stained diaper or some grossly bloody (red) stool with a suitable
amount of water to obtain a distinctly pink supernatant hemoglobin solution; (2)
centrifuge the mixture and decant the supernatant solution; (3) add 1 part of 0.25 N
(1%) sodium hydroxide to 5 parts of the supernatant fluid. Within 1-2 min, a color
reaction takes place: A yellow-brown color indicates that the blood is maternal in
origin; a persistent pink indicates that it is from the infant. A control test with known
adult or infant blood, or both, is advisable.
57(8). Long cords (>70 cm) increase risk for true knots, wrapping around fetal parts
(neck, arm), and/or prolapse. Straight untwisted cords are associated with fetal
distress, anomalies, and intrauterine fetal demise.
58.(C). Most umbilical hernias that appear before the age of 6 mo disappear
spontaneously by 1 yr of age. Even large hernias (5-6 cm in all dimensions) have been
known to disappear spontaneously by 5-6 yr of age.
59(8). Persistent edema of 1 or more extremities may represent congenital
lymphedema (Milroy disease) or, in females, Turner syndrome.
60(A). Diabetic mothers have a high incidence of polyhydramnios, preeclampsia,
pyelonephritis, preterm labor, and chronic hypertension; their fetal mortality rate is
greater than that of nondiabetic mothers, especially after 32 wk of gestation. Fetal loss
throughout pregnancy is associated with poorly controlled maternal diabetes
(especiallyketoacidosis) and congenital anomalies.
81(8). The probable pathogenic sequence is that maternal hyperglycemia causes fetal
and the fetal pancreatic responseleadsto fetal hyperinsulinemia;fetal
hyperglycemia,
173
hyperinsulinemia and hyperglycemia then cause Increased hepatic glucose uptake and
glycogen synthesis, accelerated Ilpogenesls, and augmented protein synthesis. Related
pathologic findings are hypertrophy and hyperplasia of the pancreatic islet [3 cells,
increased weight of the placenta and Infant organs except for the brain, myocardial
hypertrophy, increased amount of cytoplasm In liver cells, and extramedullary
hematopoiesis. The incidence of congenital anomaliesis Increased 3-fold in infants of
diabetic mothers; cardiac malformations (ventricular or atrial septal defect,
transposition of the great vessels, truncus arteriosus, doubieoutlet right ventricle,
tricuspid atresia, coarctation of the aorta) and iumbosacral agenesis are most
common.
174
71.(C).
of
72.(E).Pneumoniaand empyema are common respiratory tract manifestations
neonatal bacterial infections.
73.(D)tBreastabscessand impetigo are common skin and soft tissue manifestationsof
neonatal bacterial infections.
74(3). Abnormal white blood cell (WBC) count or an increase in immature forms is
included in the definition of the systemic inflammatory response SIRSin adults.
7S.(D). Staphylococcuscoagulase negative (9.1%), Group B streptococcus (21.9%),
Escherichiacoli (34.0%), Pseudomonas (74.4%), and Candida albicans (43.9%).
76.(C).The umbilical cord generally dries up and sloughs by 2 weeks of life. Delayed
separation can be normal up to 45 days. However, because neutrophilic and/or
monocytic infiltration appears to play a major role in autodigestion, persistence of the
cord beyond 30 days should prompt consideration of an underlying functional
abnormality of neutrophils (leukocyte adhesion deficiency) or neutropenia.
77.(E). In an analysis of 15,072 neonates born at 98 centers over a 2-year period, the
most important variables associated with NEC were gestational age and birth weight.
78.(E). Pneumatosis intestinalis can be seen in various other conditions, including
Hirschsprung's disease, pseudomembranous enterocolitis, neonatal ulcerative colitis,
and ischemic bowel disease. However, it is a characteristic finding in 85% of patients
with NEC. Dark, concentric rings within the bowel wall represent hydrogen as a
byproduct of bacterial metabolism
79.(B). Reticulocytosis (>8% at birth, >S% during first 2-3 days, >2% after first week).
80.(D). Reducing sugars (e.g., glucose, fructose, galactose, pentoses, and lactose) are
detected, but sucrose is not a reducing sugar.
81.(C). Chvostek's sign (facial muscle twitching on tapping), and Trousseau's sign
(carpopedal spasm) may be present, but more commonly these are absent during the
neonatal period.
82.(D). In a series of infants studied by ultrasonography, approximately 50% had the
onset of hemorrhage on the first day of life, 25% on the second day, and 15% on the
third day. Thus, a single scan on the fourth day of life would be expected to detect
>90% of lVHs.
83(8). The antenatal administration of magnesium sulfate before preterm delivery has
been suggested to decrease the risk of cerebral palsy in surviving infants but not
improve overall survival. Prospective multicenter randomized trials are currently
examining this potential benem.
175
dolescent edicine
QUESTIONS
YDER ALMUSAWI
1. Adrenal of androgen
production odorandfaint
of underarm
withdevelopment
mayoccurasearlyas
genitalhair (adrenarche)
A. 4 yr
8. 5 yr
C. 6 yr
D. 7 yr
E. 8 yr
2. In males, the first visible sign of puberty is testicular enlargement, beginning as early
as
8.5 yr
9.5 yr
wpnwr 10.5 yr
11.5 yr
12.5 yr
178
mavisible
6'The 0f puberty
sign infemales
istheappearance
ofbreast
buds
between
(thelarche)
A 5 and 9 yr
B. Sand 10 yr
C. 7 and 11 yr
D. 8 and 12 yr
E. 9 and 13 yr
11.Which
of thefollowing
scores according
asserious of
to theassessment
regarded
seriousness
of adolescent drug abuse score?
177
A. 0-3
B. 3-8
C. 813
0. 13-18
E. 18-23
male
A13-years-old
12. presented mumbling
and
delirium
with
flushed dilated
skin, myoclonus,
pupils,
Speechttizhycard
u , urinary
tempera
elevated
slightly
dry seizure
lateronhedeveloped and
anddecreased
retention, sounds,
bowel
dysrhythmia.
the MOSTlikelycauseis
0f thefollowing,
A. antidepressant agents
B. amphetamine
C. barbiturates
D. benzodiazepines
E. edrophonium
W909?
15. Exposure
300g
400g
500g
to smokeless
tobaccoincreases
the usersriskfor
A lipoid pneumonia
8. chronic cough
C. cancersof the esophagus
D. irritability
E decreased concentration
178
A. dizziness
B. miosis
C. nausea
D. flushing
E. hyperthermia
19. Evaluationfor pubertal delay in female should be done if she lacks any pubertal
signsbythe age of
A. 12 yr
B. 13 yr
C. 14 yr
D. 15yr
E. 16 yr
21'Primary
amenorrhea evaluation,
requires
generally doesnotoccur
ifmenstruation
within
A- 1yrfromtheonsetof
puberty
3- 2yrfromtheonsetof
puberty
179
C. 3 yr fromthe onsetof puberty
D. 4 yr fromthe onsetof puberty
E. 5 yr fromthe onsetof puberty
22.TheMOST used
commonly is
forcontraception
method
A. condom
withdrawal
990!
oral contraceptive pill
spermicides
intrauterine device
23.Thecontraceptive
method failurerateevenwithperfectuseis
withthehighest
A. progestin releasingintra uterine device
8 progestin~only injection
C. combined oral contraceptives
D. male condom
E. spermicides
PPS? acne
weight gain
osteoporosis
180
Adolescent Me .,icine
ANSWERS
yAYoERALMUSAWI
1.(C).Adrenal production of androgen (chiefly dehydroepiandrosterone sulfate
[DHEASD may occur as early as 6 yr of age, with development of underarm odor and
faint genital hair (adrenarche).
2(8), 3.(C), 4.(D), 5.(C). In males, the first visible sign of puberty and the hallmark of
SMR 2 is testicular enlargement, beginning as early as 9.5 yr, followed by the
development of pubic hair. This is followed by penile growth during SMR 3. Peak
growthoccurswhen testis volumesreachapproximately9-10cm3duringSMR4. Under
the influence of luteinizing hormone and testosterone, the seminiferous tubules,
epididymis, seminal vesicles, and prostate enlarge. Sperm may be found in the urine by
SMR 3; nocturnal emissions may be noted at this time as well.
6(0), 7(0). in females, typically the first visible sign of puberty and the hallmark of
SMR 2 is the appearance of breast buds (thelarche), between 8 and 12 yr of age. A
significant minority of females develops pubic hair (pubarche) prior to thelarche. Less
visible changes include enlargement of the ovaries, uterus, labia, and clitoris, and
thickening of the endometrium and vaginal mucosa. A clear vaginal discharge may be
present prior to menarche (physiologic leukorrhea). Menses typically begins 2.5 yr after
the onset of puberty, during SMR 3-4 (average age: 12.5 yr; normal range: 9-15 yr).
8.(B). This maturation is characterized by decreases in gray matter, increases in white
matter, and an apparent increase in the efficiency of communication and connectivity
between different brain regions.
9.(C). On examination, the classic finding is mucoid or purulent discharge from the
urethral meatus. if no discharge is evident on exam, providers may attempt to express
discharge by applying gentle pressure to the urethra from the base distally to the
meatus 34 times.
181
1418). Smoking during pregnancy is associated with an average decrease in fetal
Weight of 200 g; this decrease, added to the already smaller size of infants born to
teenagers, increases perinatal morbidity and mortality.
15.(C). The 2 forms of smokelesstobacco (SLT)are chew, a leafy tobacco product sold
in pouches, and snuff, a finely ground tobacco product sold in tins or packets.
Exposure to SLT increases the users risk for oral cancers of the mouth, pharynx, larynx,
and esophagus, as well as gum disease and nicotine addiction.
16.(B). Mydriasis.
11(3). Patient in the question presented with sympathomimetic syndrome and this
syndrome can be caused by cocaine, amphetamine, methamphetamine, and OTC
decongestants (phenylpropanolamine, ephedrine, and pseudoephedrine). In caffeine
and theophylline overdoses, similar findings, except for the organic psychiatric signs,
result from catecholamine release.
18.(D). Occurring in up to 60% of normal adolescent males, onset typically is between
10 and 13 yr, usually regresses within 18-24 mo, no medical therapies for gynecomastia
have been approved for use in adolescents.
19.(B).
20.(D), 21.(D). Age 15 yr is commonly considered the age at which an evaluation for
primary amenorrhea should be undertaken. Evaluationshould begin sooner if 4 yr have
elapsed since the onset of puberty (breast development in most females).
22.(Ai. The most commonly used method is the condom, followed by withdrawal and
then the pill.
23.(E). Failure rate are as follow: Progestin releasing intra uterine device 0.2%,
progestin-only injection 0.2%, combined oral contraceptives 0.3%, male condom 2%,
Spermicides 18%.
24.(E). After 1 yr of use, 50% of users develop amenorrhea, which may be an added
advantage for teens with heavy menstrual bleeding, dysmenorrhea, anemias, or blood
dyscrasias.Although concern has been directed toward the potential for loss in bone
mineral density in adolescents,thereby potentially increasingtheir risk for osteoporosis
later in life, subsequent studies have found that bone density is recovered after
discontinuation of the method and is considered safe for use in this population.
182
mmuno ogy
QUESTIONS
1.A94nonth-0ld
bOY["856"th Withrecurrent
attaCks
ofdiarrhea, andfailure
thrush,
tothriveshortlyafter birth.Yoususpectimmunedeficiency.
of the following, the MOST commoncauseof this conditionis
A. hyperIgE syndrome
8. defect in phagocytic cells
C. deficiencies in T-cell function
0. defect in antibody production
E. defect in complement proteins
W909?lgM measurement
lgE measurement
isohemagglutinins titer
4. A 3-year-old
boypresentedwith recurrent andotitismedia
attacksof pneumonia
sincethe1 birthday. isX-linked
Yourdiagnosis agammaglobulinemia.
0f thefollowing,the MOSTlikelyoffendingorganismis
A. CMV
3- Mycoplasma
C. Pneumocystisjiroveci
9- Staphylococcus
aureus
E. Streptococcus
pneumonia
183
5. A 45-year-old boy presented with history of recurrent attack of secretary otitis
media and purulent nasal discharge, Haemophilus influenza revealed by culture of ear
discharge, on physical examination there is no tonsillar tissue and no palpable lymph
nodes.
Of the following, the MOST appropriate test to confirm the diagnosis is measurement
of
flow cytometry
lgA concenteration
W909?isohemagglutinins titer
IgG and lgM concenteration
antibodies to antigens of routine immunizations
6. A 3year-old girl presented with recurrent attacks of chest infection and frequent
episodes of chronic diarrhea, stool examination revealed Giardia lamblia and serum IgA
level was very low.
Regarding this condition, all the following are true EXCEPT
A. autosomal dominant inheritance
B. phenotypically normal blood B cells
C. administration of IVIG is not indicated
D. environmental factors may trigger the disease
E. it is associated with a celiac-like syndrome with dramatic response to gluten-
free diet
7. A 2-year-old boy presented with failure to thrive, neonatal hypocalcemic seizure, and
three attacks of sepsis. Examination reveals cleft palate and holosystolic murmur.
Of the following, the genetic predisposition of this condition is
A. microdeletions of chromosome 22q11.2
B. interstitial deletion of the long arm of chromosome 15 (15q11-13)
C. presence of two copies of paternally derived chromosome 11p15.5
D. presence of mutated gene on long arm of chromosome 11 (11q22-23)
E. presence of abnormal gene, on the proximal arm of the X chromosome at
Xp11.22-11.23
8. 0f the following, the findings that are MOST suggestive of DiGeorge syndrome are
A. cataract and a congenital heart defect
B. hypotonia and a congenital heart defect
C. cutis aplasia and a congenital heart defect
D. hypocalcemia and a congenital heart defect
E. mongoloid slant to the eyes and a congenital heart defect
184
A. vaccination
B. administration of MG
C. judicious use of antibiotics
D. transplantation of thymic tissue
E. transplantation of hematopoietic stem cells
F190!
protracted bloody diarrhea
EBV-associated malignancies
development of autoimmune disease
trauma
aggressive antibiotics
administration of MG
bone marrow or cord blood transplantation
14.Severe count
eosinophil
asanabsolute
defined (AEC) than
more
eosinophilia,
A. 750cells/uL
B. 1,000 cells/pL
C. 1,500 cells/uL
D. 5,000 cells/uL
E. 7,500 cells/pl
185
isTRUE
statements
of thefollowing
15.Which diurnal
regarding variation
0f the
absoluteeosinophilcount(AEC)?
higher in the evening
lower in the afternoon
POP?
higher in the afternoon
lower in the early morning
higher in the early morning
19.Severe isdefined
neutropenia asabsolute count(ANC)lessthan
neutrophil
A. SOD/pd.
B. 750/uL
c. woe/lit
o. 1250/uL
E. 1500/uL
20.Chronic
neutropenia
bydefinition
lastslonger
than
A. 1 mo
8. 3mo
C. 6mo
D. 9mo
E. 12 mo
186
21.Chronic maybe induced
neutropenia by
A. adenovirus
B. enteroviruses
C. cytomegalovirus
D. influenzas A and B
E. respiratory syncytial virus
POP
Staphylococcus aureus and Cytomegalovirus
Staphylococcus aureus and Pneumocystis carinii
Staphylococcus aureus and gram-negative bacteria
ear'0 boy
25-A 15-Y rheu
With matoidarthritis,on quininefor 6 months, presented
.
Withfever,aphthous andfatiguefor the
stomatitis, 5
last days. Hisabsoluteneutrophil
mm (ANC)was700/uL
TileMOST
nextappropriate measure
therapeutic to bedonewhenquininewithdrawal
failsto irnproveneutropeniais
A- administration of MG
3- aggressiveuse of antibiotics
stemcells
ofhematopoietic
transplantation
:-- administration
of pulsemethylprednisolone
ulatin gfactor
-St'm
E. administration human
ofrecombinant C°'°"Y
granulocvte
187
26. A 9-year-oldboy withacutelymphoblastic leukemia on chemotherapy,presented
with fever,aphthousstomatitis,andfatiguefor the last5 days.HisabsoluteneutrOphiI
count (ANC)was450/uL and hemoglobinlevelwas 10gm/dl.
The NEXT appropriate therapeutic measure is
A. administration of MG
8. transplantation of hematopoietic stem cells
C. administration of pulse methylprednisolone
D. aggressive use of broad-spectrum antibiotics
E. administrationof recombinanthumangranulocytecolony-stimulatingfactor
acne?drug-induced neutropenia
acute lymphoblastic leukemia
Shwachman-Diamond syndrome
28. Leukemoid reactions (WBC count > 50,000/uL) are characterized by abundant
blasts
band form
91.009"?
myelocytes
promyelocytes
metamyelocytes
29. Leukemoid reactions (WBC count >50,000/uL) are caused by all the following
EXCEPT
A. shigellosis
B. septicemia
C. salmonellosis
D. chronic granulomatous disease
E. leukocyte adhesion defect with infection
PP!
hemorrhage
acute hemolysis
megaloblastic anemia
188
31. A 15-year-oldboy presentedwith high fever, lymphadenopathy,
recurrent
hepatosplenomegaly, skininfections, hair,andphotOphobia.
silvery Last
year,
ataxia.
hedeveloped
findingin thiscondition
TheMOSTcharacteristic is
A. neutropenia
thrombocytopenia
prolongedbleedingtime
r1905:
prolongedprothrombinetime
prolongedpartial prothrombinetime
33.A 3-year-old
boypresented subcutaneous
withrecurrent abscesses,
Iymphadenitis,
and family history of recurrent infections.
osteomyelitis,
The hallmark of this condition is
A. hypocalcemia
B. atopic dermatitis
thrombocytopenia
34.TheMOST
common inchronic
pathogen granulomatous (C60)is
disease
A. S. aureus
B. Aspergillus
C. Salmonella
D. Mycobacterium
E. Candida albicans
35-
The ofchronic disease made
often
C60isMOST W
diagnosis granulomatous
A. DNAanalysis
. flow cytometry
189
number
the infection
ofbacterial chronic
with
inpatients granulomatous
reduce
36To daily
begiven
disease should
they
(CGD),
A. penicillin
B. cloxacillin
C. interferon-y
D. itraconazole
E. trimethoprim~sulfamethoxazole
disease method
thebest
(CGD): and
todetectfollow
Up
Inchronic
37. granulomatous
deepseatedinfectionis
ESR
blood culture
W905?WBC count
Cereactiveprotein
immatureto matureneutrophilratio
inproperdin
arecharacteristic
38.Allthefollowing EXCEPT
deficiency
dermal vasculitis
male predominance
W995?systemiclupus erythematosus
susceptibilityto N. meningitidismeningitis
normal serum hemolytic complement activity
39. All the following matching about genetic deficiencies of plasma complement
components and associatedclinicalfindings are true EXCEPT
A. C4 -- discoid lupus erythematosus
B. C1q -- systemic lupus erythematosus
C. C1 INH hereditary angioedema
D. factorH- atypicalhemolytic-uremicsyndrome
E. properdin-- N. meningitidis
meningitis
40. ofboth
component theclassical
andalternative is
Ireamplement pathways
B. C3
C. C4
D. C5
E. C6
41. Allthe
followingcondition
s can induce
A. sepsis lymphopenia EXCEPT
3- typhoid
C. brucellosis
190
D. corticosteroiduse
E. cartilagemairhypoplasia
191
lmmuno ogy
ANSWERS
l9.
1.(C). Children with defects in antibody production, phagocytic cells, or complement
proteins have recurrent infections with encapsulated bacteria and may grow and
develop normally despite their recurring infections, unless they develop bronchiectasis
from repeated lower respiratory tract bacterial infections or persistent enteroviral
infections of the central nervous system. By contrast, patients with deficiencies in T-cell
function usually develop opportunistic infections or serious illnesses from common
viral agents early in life, and they fail to thrive.
2.(A). If the lgA level is normal, selective lgA deficiency, which is the most common B~
cell defect, is excluded, as are most of the permanent types of
hypogammaglobulinemia, as is
lgA usually very low or absent in those conditions. if lgA
is low, lgG and lgM should also be measured.
3.(C). There is a possible loss of immunogiobulins through the urinary or
gastrointestinal tracts (nephrotic syndrome, protein-losing enteropathies, intestinal
lymphangiectasia).
4.(F.). They acquire infections with extracellular pyogenic organisms, such as
Streptococcus pneumoniae and Haemophilus influenzae, unless they are given
prophylactic antibiotics or immunogiobulin therapy.
5.(A). The diagnosis of X-linked agammaglobulinemia (XLA) should be suspected if
lymphoid hypoplasia is found on physical examination (minimal or no tonsillar tissue
and no palpable lymph nodes). Flow cytometry is an important test to demonstrate the
absence of circulating B cells,which will distinguishthis disorder from common variable
immunodeficiency,the hyper-lgM syndrome, and transient hypogammaglobuiinemia of
infancy.
6.(E). An isolated absence or near absence (<10 mg/dl.) of serum and secretory lgA is
the most common well-defined immunodeficiency disorder. lgA deficiency is associated
with a celiac-like syndrome, which may or may not respond to a gluten-free diet.
7.(A). A. DiGeorge syndrome, 8. PraderWilli syndrome, C. Beckwith-Wiedemann
syndrome, D. Wiskott-Aldrichsyndrome, and E. ataxia-telangiectasia.
8.(D). Digerogesyndrome;associatedwith congenital heart disease (conotruncal, atrial,
and ventricular septal defects). The diagnosisis often first suggested by hypocalcemic
seizuresduring the neonatal period.
9.(D).
10.(D). WiskottAldrich syndrome, an Xlinked recessivesyndrome, is characterized by
atopic dermatitis, thrombocytopenlcpurpura with normal-appearing megakaryocytes
but small defective platelets,and undue susceptibilityto infection. Survival beyond the
192
teens is rare; infections, bleeding, and EBV-associated malignancies are major causes of
death.
11.(E). Treatment; Good supportive care includes appropriate nutrition, routine IVIG,
use of killed vaccines, aggressive management of eczema and associated cutaneous
infections, platelet transfusion for serious bleeding episodes, splenectomy if a
transplant is not going to be done, and high-dose IVIG with systemic steroids for
autoimmune complications. Bone marrow or cord blood transplantation is the
treatment of choice and is usually curative.
12.(A). The most prominent clinical features are progressive cerebellar ataxia,
oculocutaneous telangiectasias, chronic sinopulmonary disease, a high incidence of
malignancy, and variable humoral and cellular immunodeficiency.
13.(A). Ataxia typically becomes evident soon after these children begin to walk and
progressesuntil they are confined to a wheelchair, usually by the age of 10-12 yr. The
telangiectasias begin to develop at 3-6 yr of age.
14.(D). Moderate (AEC 1,500-5,000 cells/pL) and severe (AEC>5,000 cells/uL).
15.(E). The absolute eosinophil count (AEC) is calculated as the white blood cell
count/uL x percent of eosinophils, it is usually <450 cells/pL and varies diurnally, with
eosinophil numbers higher in the early morning and diminishing as endogenous
glucocorticoid levels rise.
16.(C). Exogenous and endogenous glucocorticoid suppresses absolute eosinophil
count.
193
24.(B). The process of neutropenia likely arises from effects of drugs, such as
propylthiouracil or penicillin, that act as haptens to stimulate antibody formation, or
drugs, such as quinine, that induce immune complex formation. Late-onset
neutropenia can occur after rituximab therapy. Idiosyncratic reactions, for example to
chloramphenicol, are unpredictable with regard to dose or duration of use.
Hypersensitivity reactions are rare and may involve arene oxide metabolites of
aromatic anticonvulsants (phenytoin or phenobarbital).
25.05).
26.(D). A decline in the WBC c0unt typically occurs 7-10 days after administration of
the anticancer drug and may persist for 1-2 wk. Patients with chemotherapy/ radiation~
related neutropenia and fever must be treated aggressively with broad~spectrum
antibiotics.
27.(E). Shwachman-Diamond syndrome (505) is an autosomal recessive disorder
classically characterized by neutrOpenia, pancreatic insufficiency, and short stature
with skeletal abnormalities. The initial symptoms are usually steatorrhea and failure to
thrive because of malabsorption, which usually develops by 4 mo of age, although the
gastrointestinal symptoms may be subtle in some patients and go unrecognized.
Virtually all patients with 505 have neutropenia, with the ANC periodically <1000/uL.
28.(B). Leukemoid reactions are usually neutrophilic, and unlike true leukemia, show
only small proportions of immature myeloid cells, consisting primarily of band forms,
occasional metamyelocytes, and progressively rarer myelocytes, promyelocytes, and
blasts.
29.(D).
30.(E). Higher degrees of left shift with more immature neutrophil precursors are
indicative of serious bacterial infections and may be a direct sign of depletion of the
bone marrow reserve pool of neutrophils. Marked left shift may occasionally be
encountered with trauma, burns, surgery, acute hemolysis, or hemorrhage.
31.(C). Patients with Chédiak-Higashi syndrome (CHS) have prolonged bleeding times
with normal platelet counts, resulting from impaired platelet aggregation associated
with a deficiency of the dense granules containing adenosine diphosphate and
serotonin.
32.(A).
33.(D).Granulomaformation and inflammatory processesare a hallmark of chronic
granulomatous diseaseCGDand may be the presentingsymptoms that prompt testing
for CGDif they causepyloricoutlet obstruction,bladder outlet or ureter obstruction, or
rectal fistulas and granulomatous colitis simulating Crohn disease.
34.(A).The most commonpathogenis S. aureus,although any catalase-positive
microorganismmay be involved. .
35.(B).The diagnosisis most often made by performing flow cytometh 5'"8
123(DHR)
dihydrorhodamine oxidant
to measure throughits increased
production
fluorescencewhen oxidized by H202.
194
studyfoundthat interferon'y
35(5).A placebo-controlled reduces
significantly the
and seriousinfections.
number0f hospitalilations Itraconazole
administered
the
reduces frequency
prophylactically offungal
infections.
Theerythrocyte
37_(A). sedimentation rate(ESR) canbequitehelpful,If thechilddoes
nothavea deep-seated the
infection, ESR will be normalor willnormalizewithin
severaldayswithstandardmanagement. However, if it doesnot,a search
for deep
asisconsideration
iswarranted,
tissues of empiricantibiotics,
Typical
38.(C). discoid
lupuserythematosus iscommon.
Systemic
39.(A). lupuserythematosus (SLE) isverycommon inC4deficiency.
40. (B).
Chronic
41.(C). infections
bacterial mayleadto a
andbrucellosis
suchastuberculosis
sustained
lymphocytosis.
195
Disorders
Allergic
- QUESHONS
iiASANEINGHALI
chronic
asamajor
rhinitis
ofallergic respiratory ofchildren
disease
1.The
recognition EXCEPT
rests
largely features
onallthefollowing
high prevalence
effect on quality of life
rhinitis
ofallergic
2.Thediagnosis ageof
bythetimethechildreaches
isestablished
A. 2 yr
8. 4 yr
C. 6 yr
D. 8 yr
E. 10 yr
4. The critical period for the development of allergic rhinitis exists when the genetically
susceptible individual at greatest risk of sensitization in
A. early adolescence
8. late adolescence
C. school age
D. toddlerhood
E. early infancy
196
E. 3yr
of allthefollowingdecrease
6.Earlyintroduction theriskofasthma
andallergic
rhinitis
EXCEPT
A. rye
B. fish
C. egg
D. meat
E. wheat
8. The main differentiating item between intermittent versus persistent allergic rhinitis
'
l5
A. school performance
B. troublesome symptoms
C. daily activities
D. number of attacks per week
E. sleep pattern
bacterialinfection35sinusitis.
10.Allergicrhinitismaybe complicatedby secondary
Of the following, the MOST likely clue to infection is
A. hyperemia
B. swollen turbinates
C. thick nasal secretions
D. bluish mucus membranes
E. edematous mucus membranes
197
hypothyroidism
exercise
mpnPP
The
12.
cancer
vaculitides
of
rate
reported of
erythematosis
lupus
systemic
rhinitis
allerslc
remission uabout
children
among
110%
11-20%
wpnw?
.W hat
13
2130*:
31-40%
41-50%
rhinitis
ofallergic
percentage has conjunctivitis?
allergic
patients
A. >30%
8. >40%
C. >50%
0. >60%
E. >70%
of allergic
14. Thepercentage in asthmatic
rhinitis is 78%,whilethe
patients
withallergicrhinitisis
of asthmain patients
percentage
A. 18%
B. 28%
C. 38%
D. 48%
E. 58%
WPDP?5 day
7 day
9 day
16. Th e bestmethod
fordetection
of
A. skin
tests alierg
en- Specific
IgEis
a. serumimmune forspecific
assay IgE
C. nasalsmearstudy
D. eosinophilia in blood film
1..
Lm
E. total serum igE concentration
17.Second antihistamines
generation antihistamines
to firstgeneration
arepreferable
because they have
longer action
less sedation
20. 0f the following, the major risk factor for persistent asthma is
A. eczema
8. allergic rhinitis
C. wheezing apart from colds
0. food allergen sensitization
E. 24% peripheral blood eosinophils
21. Of the following, the strongestidentifiable factor for the persistenceof childhood
asthma is
A. allergy
B. male gender
C. low birth weight
D. parental asthma
E. lower respiratorytract infection
22. The following actions may help reduce the likelihood of asthma development
EXCEPT
A. healthy diet
199
immunization
. activelifestyle
99? of tobacco
avoidance
prolonged
smoking
>4 months
breastfeeding
children.
ofpreschool-age
23.Recurrent and
wheezing
coughing In35%
occurs
Those
who tohave
continue asthma
persistent areapproximately
intolaterchildhood
A. one-fifth
B. one-fourth
C. one-third
D. one-half
E. two-thirds
of asthmaincludethe followingEXCEPT
of severeexacerbation
24. indications
A. breathlessness
accessorymusclesuse
wpnw
labored breath
PEFor FEV1value <70% of personal best values
mental status changes
A. normal
physical
activity
5. PEF
>70%
ofpredicted
orpersonal
best
200
C. exercise tolerance
saturation
D. oxygen roomairfor4 hr
>92%whilethepatientisbreathing
E. no more use of accessory muscles
28.Allthefollowing
areconsidered forasthma
asriskfactors andmortality
morbidity
EXCEPT
A. poverty
B air pollution exposure
C. female gender
D. poor response to systemic steroid therapy
E. sudden asphyxia episodes
29. The best "rescue" medication in the treatment of acute asthma symptoms is
A. oral SABA
B. inhaled SABA
C. oral corticosteroid
D. inhaled ipratropium
E. inhaled corticosteroid
30. The best treatment option for step -6- severe persistent asthma in a 6year-old boy
IS
201
D. 215%
E. 218%
'
inPeak
variation
diurnal Flow
(PEF)
Expiratory Is
that cons'Ste
nt with asthma - is
33.The
more than
A. 10%
20%
WP?!
30%
40%
50%
35. All the following are a recognized features of a "well-controlled asthma in a 7'
year-old boy EXCEPT
A. FEVltFVC ratio >80%
8. FEV10f >80% of predicted
C. daytime symptoms 52 days/wk
D. 24 exacerbations in the past
year
E. need a rescuebronchodilator $2
days/wk
36. ofastoplight zone
system
(green,
Lise
best PEF
yellow,
red)tailoredto eachchild'spersonal
valuescan optimizeeffectiveness
andinterest.
0f the following,the PEFvaluein
yellowzoneis
A. <10%
8. 10-30%
C. 30-50%
D. 50-80%
E. 8010096
202
B. omalizumab
C. montaleukast
D. inhaled corticosteroid
E. sustained-release theophylline
39. Atopic dermatitis affects many children worldwide, the exact percentage
approximating
A. 5-10%
B. 10-30%
C. 30-50%
D. 50-70%
E. 70-90%
91.005?
high humidity
herpessimplex
excessivesweating
44.ininfancy, dermatitis
atopic and
acute
isusually the
spares
oftheextremities
surfaces
A. extensor
B. forehead
C. scalp
D. diaper area
E. cheeks
children
45.Older chronic
with have
dermatitis
atopic and
lichenification to
atendency
be localized to
flexural folds of the extremities
forehead
WP???scalp
diaper area
cheeks
090??relapsing dermatitis
facial eczema in infants
extensor eczema in adolescents
48. One of the following metabolic c ' ' ' ' '
_ _ onditions IS n 0t deemed
m thediffere
ntlal
diagnOSIs dermatitis
ofatopic
A. zincdeficiency
B. pyridoxine
deficiency
C. cobalamin
deficiency
D. niacindeficiency
204
E. phenylketonuria
999$? antihistamine
tar preparations
topical corticosteroids
50. Prevention of atopic dermatitis in infancy includes the following measures EXCEPT
breast feeding
feeding with a hypoallergenic hydrolyzed formula
51. Which of the following vitamin deficiencies often accompanies severe atopic
dermatitis?
A. E
B C
C A
D. D
E 812
52. Predictive factors of a poor prognosisfor atopic dermatitis includesall the following
EXCEPT
A. widespread atopic dermatitis in childhood
8. filaggrin gene null mutations
C. concomitant allergic rhinitis and asthma
D. family history of atopic dermatitis in parents or siblings
E. late age at onset of atopic dermatitis
205
A. head and neck
B. hands and arms
C. legs and feet
D. upper chest
E. lower abdomen
56. One of the following medications used in treatment of atopic dermatitis should be
discontinued after failure to achieve good results within 4-6 weeks
A. methotrexate
B. azathioprine
C. cyclosporine
D. mycophenolate mofetil
E. omalizumab
57. Systemiccorticosteroids
are rarely indicatedin the treatment of atopic dermatitis
because
A. toxic side effects after long term use
8. rebound flare after therapy discontinuation
C. cannot do more than what topical can
D. tapering is required even after short term use
E. antimetabolitescando better in
modifyingdiseasecourse
58.Theleastpotenttopicalsteroidusedintreatmentof
atopicdermatitisis
A. fluticasone
B. desonide
C. betamethasonedipropionate
D. hydrocortisone
E. clobetasolpropionate
topical
steroid
i5Preferable
touse
fortreatment
ofatopic inthe
dermatitis
fix/mm
206
fluticasone 0.005%
desonide 05%
Trichophyton rubrum
Molluscum contagiosum
61. Exfoliative dermatitis may develop in patients with atopic dermatitis and extensive
skin involvement, usually caused by inappropriate therapy or superinfection with
A. E. coli
B. Herpes zoster
C. Herpes simplex
D. Streptococcal pyogen
E. Trichophyton rubrum
207
E. yellow jackets
65.Delayed/Late
reaction allthefOI'OngEXCEPT
venommayprecipitate
tostinging
A. vasculitis
8. serum sickness
C. encephalopathy
D. hemolytic anemia
E. nephrotic syndrome
66.TheMOSTreliablediagnostic fordetection
modality IgEis
of venom-specific
A. in vitro serum assayfor venom-specific IgE
8. skin test
C. serum tryptase level
D. plasma histamine
E. radioallergosorbent assay
67. In the presence of convincing history of a severe systemic reaction, the next
diagnostic step for those with initially negative skin test is
in vitro serum assay for venom-specific IgE
repeat skin test after 4-6 weeks
F1905?
serum tryptase level
plasma histamine
basophil histamine release test
epinephrine
WP???antihistamines
corticosteroids
intravenous fluids
208
generalizedcutaneousreactionwith positiveskintest and negativein vitro test
generalizedcutaneousreactionwith positivein vitrotest andnegativeskintest
mpnw systemic reaction with positive skin test and negative in vitro test
systemic reaction with negative skin test and negative in vitro test
71. Skeeter syndrome is a large local reaction to stinging and biting insects.
Of the following, the TRUE statement is
lg E mediated response
usually occurs in older children
meow?
atopic keratoconjunctivitis
giant papillary conjunctivitis
contact allergy
209
76.Topical
ophthalmic usuallycause
medications
A. contact allergy
B. allergicconjunctivitis
C. vernal keratoconjunctivitis
D. atopic keratoconjunctivitis
E. giant papillary conjunctivitis
80. Chronic
urticaria
maybecausedby
A. latex
B. peanut
C. lVimmunogiobulin
D. streptococcal
pharyngitis
E. systemiclupus
erythematosus
81. Thedrugof choicefor
cold-in
ducedurticariais
A. loratadine
B. epinephrineW
C. cyproheptadine
D.
diphenhydramine
E. oral
corticosteroid
210
82. Acute urticaria is a self-limited illness requiring little treatment. All the following
can be used EXCEPT
A. loratadine
B. hydroxyzine
C. epinephrine lM
D. H2 antihistamines
E. oral corticosteroid
83. Skin biopsy for diagnosis of possible urticarial vasculitis is recommended for
A. urticarial lesions that persist at different locations for >24 hr
B. those with non- pigmented or non-purpuric components
C. those that burn more than itch
D. those with associated collagen vascular diseases
E. those appeared after peanuts ingestion
84. The differential diagnosis of chronic urticaria includes the following EXCEPT
A. cutaneous mastocytosis
B. systemic mastocytosis
C. complement- mediated mast cell degranulation in malignancies
D. cutaneous blistering disorders
E. dermatographism
211
ina prevrously
symptoms
ofcutaneous
inthepresence healthy
88.Sudden
collapse
of
child shouldraisesuspicion
A. vasovagalcollapse
anaphylaxis
wpnp
aspiration
pulmonaryembolism
seizure disorder
89.Cutaneous maybeabsent
symptoms in
inanaphylaxis
5%
10%
WP???20%
30%
40%
FIPOP?
radioallergosorbent assay
plasma tryptase
immunoCAP lgE test
8. meropenem
212
C. rituximab
D. trimethoprim sulfate
E. carbamazepine
95.
none? 5-8 weeks
9-12 weeks
13-18 weeks
is unlikelyin serumsickness?
hichof the followingresultsof investigations
elevated erythrocyte sedimentation rate
POPPE
thrombocytosis
reduced C3 activity
negative microbial cultures
microscopic hematuria
96. Skin biopsy is not usually necessary for confirming the diagnosis of serum sickness
because
A. the complement C3 and C4yield better results
B. the clinical examination is enough for the diagnosis
C. skin test is superior to biopsy
D. the histopathological findings are not specific
E. it may be complicated by local infection
98. All the followingare symptomsof acute lgEmediated food allergy EXCEPT
A. urticaria
B. pruritis
C. abdominal pain
D. diarrhea
E. wheezing
213
A. Heiner syndrome
anaphylaxis
B. gastrointestinal
C. rhino conjunctivitis
D. oral allergysyndrome
E. angioedema
100.Theonset offoodallergyislatein
ofpresentation
A. hen's egg white
8. peanuts
C. shellfish
D. soybean
E. cow's milk
101.Foodprotein-induced
enterocolitis
syndrome by all the following
ischaracterized
EXCEPT
A. manifests in the first months of life
8. vomiting occurs 1-3 hours after feeding
C. hypotension occurs in approximately 15% of cases
0. commonly improved with ingestion of soy protein based formula
E. usually resolve by the age of three years of life
103.TheMOST severeformoffoodprotein~induced
A. celiac disease
is
enteropathy
8. eosinophilicesophagitis
C. oral allergysyndrome
0. acutegastrointestinal
allergy
E. food proteinproctocolitis
reactions?
A. 15%
B. 25%
C. 35%
D. 45%
E. 55%
214
in childrenwith egg
of the followingvaccineis contraindicated
105.Administration
allergy
MMR
influenza
P905? DPT
yellow fever
rabies
108. Which of the following drugs can cause Stevens - Johnson syndrome?
dapsone
hydralazine
POP? sulfonamide
procainamide
bleomycin
109. Which one of the following agents is implicated in causing cutaneous lupus?
corticosteroids
calcium-channel blockers
POP? allopurinol
B-lactam
sulfonamide
antibiotics
215
drug-induced
ofthefollowing
111One (aims isnotmdUdEd
allergic asan
indictment of allopurinol
A. exanthem
B. Stevens-Johnsonsyndrome
C. toxic epidermal necrolysis
D. pulmonaryfibrosis
E. interstitial nephritis
Rash
112.Drug andSystemic
withEosinophilia Symptoms canbeCBUSEd
(DRESS) byall
the following EXCEPT
A. anticonvulsants
sulfonamides
W905
B-lactam antibiotics
minocycline
allopurinol
.mPDPPhydrochlorothiazide
allopurinol
nonsteroidal anti-inflammatory
A. isoniazid
B. sulfonamide
C. penicillamine
D. calcium channel blockers
E. tetracycline
.mPOP?
dosing schedule
genetic predisposition
atopy
216
D. ceftazidime
E. ceftriaxone
217
m Disorders
Allergic
ANSWERS
ASANEIN GHAL
218
20.(A). Early childhood risk factors for persistent asthma have been identified and have
been described as major (parent asthma, eczema, inhalant allergen sensitization) and
minor (allergic rhinitis, wheezing apart from colds, 24% peripheral blood eosinophils,
food allergen sensitization) risk factors.
21.(A).
22.(B).
23.(C). Of these, approximately onethird who continue to have persistent asthma into
later childhood, and approximately two-thirds improve on their own through their teen
years.
24.(D). Severe airflow limitation (PEFor FEV1value <50% of personal best or predicted
values).
25.(B).
26.(A). Supplemental oxygen is the best to start with.
27.(C).
28.(C). Male gender.
29.03). Inhaled SABA, up to 3 treatments in 1 hr. If the child has an incomplete
response to initial treatment with rescue medication (persistent symptoms and/or a
PEF value <80% of personal best), a short course of oral corticosteroid therapy
(prednisone 1-2 mg/kg/day [not to exceed 60 mg/day] for 4 days), in addition to
inhaled B-agonist therapy, should be instituted.
30.(D).
31.(A). Peribronchial thickening and flattening of diaphragm.
32.(B). In asthmatic patients, an improvement in FEV1212% or >200 mL in response to
bronchodilator is consistent with asthma, while FEV1 typically decreases during or after
exercise by >15%.
33.(B).
34.(D). Discharge medications include administration of an inhaled B-agonist up to
every 3-4 hr plus a 3-7 day course of an oral corticosteroid.
35.(D). Children with wellcontrolled asthma have daytime symptoms 52 days/wk and
need a rescue bronchodilator $2 days/wk; an FEV10f >80% of predicted (and an FEV1:
FVC ratio >80% for children 5-11 yr of age); no interference with normal activity; and <2
exacerbations in the past year.
36.(D). The green zone (80.100% of personal best) indicates good control; the yellow
zone (50-80%) indicates less-than-optimal control and necessitates increased
awareness and treatment; the red zone (<50%) indicates poor control and greater
likelihood of an exacerbation, requiring immediate intervention.
37.(A). Long-term controller medications are inhaled corticosteroid, LABAs, leukotriene
modifiers, sustained-release theophylline, and nonsteroidal antiinflammatory agents.
An anti-lgE preparation, omalizumab (Xolair), is approved by the FDA for use as an add-
on therapy in children 212 yr that have moderate to severe allergic asthma that is
difficult to control.
38.(E).
219
39.(B).
40.(E).The hallmarkof atopic dermatitisis dry skin, is resultsfrom compromise of the
epidermal barrier, which leads to excess transepidermal water loss, allergen
penetration, and microbial colonization.
41.(A). Approximately 50% of patients experience symptoms in the lst yr of life, and an
additional 30% are diagnosed between 1 and 5 yr of age.
42.(D). Intense pruritus, especially at night, and cutaneous reactivity are the cardinal
features of atopic dermatitis.
43.(C). Foods(cow milk, egg, peanut, tree nuts, soy, wheat, and shellfish), aeroallergens
(pollen, grass, animal dander, and dust mites), infection (staphylococcus, herpes
simplex, and molluscum), reduced humidity, excessive sweating, and irritants (wool,
acrylic, soaps,toiletries, fragrances,and detergents) can exacerbate (trigger) pruritus
and scratching.
44.(D).
45.(A).
46.(E).The major clinical features in atopic dermatitis are (pruritus, facial and extensor
eczema in infants and children, flexural eczema in adolescents, relapsing dermatitis and
personal or family history).
47.(C).Histiocytosis,other mentioned disordersare associatedwith infections.
48.(C).
49.(A). Becausepatients with AD have impaired skin barrier function from reduced lipid
levels,they present with diffuse, abnormally dry skin, or xerosis. Moisturizers are first-
line therapy.
SO.(E).
51.(D). Vitamin D deficiencyoften accompaniessevere AD. Vitamin D enhances skin
barrier function, reduces corticosteroid requirements to control inflammation and
augmentsskinantimicrobialfunction.
52.(E).
53.(C).Erythromycinand azithromycin are usually beneficial for patients who are not
colonizedwith a resistantS.aureusstrain; a first-generation cephalosporin (cephalexin)
is recommendedfor macrolide-resistantS. aureus. Topical mupirocin is useful in the
treatment of localizedimpetiginouslesions,with systemic antibiotics for widespread
infections.
54.(A).There has been particular interest in the role of Malassezia furfur in atopic
dermatitisbecauseit is lipophilicyeast commonlypresent in the seborrheic areasof
the skin.
55.(£).Forthe other stems;A- high predictive value, 8- Positive skin test often do not
correlatewith clinicalsymptoms,0 Rarelyrequired, and D- The majority react to less
than 3 foods.
56(0),
220
57.(B). The dramaticclinicalimprovement that may occurwith systemiccorticosteroids
is frequently associatedwith a severe rebound flare of atopic dermatitis after therapy
discontinuation.
58.(D). Hydrocortisone is labeled as group 7 topical steroids which is the least potent
one.
59.(D). Use the least potent steroid in the face and intertriginous areas.
60.(B). Herpes simplex virus (HSV) can provoke recurrent dermatitis and may be
misdiagnosedas S. aureusinfection, which can be diagnosed by a Giemsa-stained
Tzanck smear of cells scraped from the vesicle base or by viral polymerase chain
reaction or culture.
61.(C). Exfoliative dermatitis associated with generalized redness, scaling, weeping,
crusting, systemic toxicity, lymphadenopathy, and fever, and is usually mused by
superinfection (e.g., with toxin-producing S. aureus or HSV infection) or inappropriate
therapy. In some cases, the withdrawal of systemic glucocorticoids used to control
severe AD precipitates exfoliative erythroderma.
62.(A).
63.(C).
64.(E). Yellow jackets are aggressiveand ground dwelling, and they linger near activities
involving food.
65.(D).
66.(B).
67.(A). in vitro serum assay for venom-specific lgE is recommended if skin test results
are negative in the presence of a convincing history of a severe systemic reaction.
68.(C).
69.(B). Adjunctive treatment includes antihistamines, corticosteroids, intravenous
fluids, oxygen, and transport to the emergency room.
70.(D). Systemicreaction at any age with positiveskintest / in vitro test is an indication
for venom immunotherapy. Large local reaction and generalized cutaneous reactions in
children are not indications for both diagnostic tests (skin and in vitro tests) and venom
immunotherapy.
71.(D). Skeeter syndrome is a large local reaction to mosquito bite, it happens usually
in young children and misdiagnosed as cellulitis. This is not an lg E mediated reaction
but because of the vasoactive and irritant material from the mosquito, it does not
cause anaphylaxis.
72.(E).
73.(A). Allergicconiunctivitisis the most common hypersensitivityresponse of the eye,
affecting approximately 25% of the general population and 30% of children with atopy.
74.(B). Vernal keratoconjunctivitis and atopic keratoconjunctivitis are sight-threatening
entities and causes visual morbidity and suspected cases need to be referred to an
ophthalmologist.
75.(B). Patients with allergic conjunctivitiscomplain of variable ocular itching, rather
than pain, with increased tearing.
221
76(A). Contact allergy typically involves the eyelids but can also involve the
Conjunctivae. It is being recognized more frequently in association with increased
exposure to topical medications, contact lens solutions, and preservatives.
77(5). Giant papillary conjunctivitis has been linked to chronic exposure to foreign
bodies, such as contact lenses, both hard and soft, ocular prostheses, and sutures.
78.(C). Topical decongestants act as vasoconstrictors; adverse effects of topical include
burning and rebound hyperemia or conjunctivitis medicamentosa with chronic use.
79.(C). Acute urticaria can also result from non~lgE~mediated stimulation of mast cells,
caused by radiocontrast agents, viral agents (including hepatitis B and Epstein-Barr
virus), opiates, and nonsteroidal anti-inflammatory agents.
80.(E). Autoimmune disease, like SLE, Juvenile rheumatoid arthritis, thyroid disease,
inflammatory bowel diseases and celiac disease, are well established causes of chronic
urticaria.
81.(C).
82(0). H2 antihistamines used in chronic urticaria.
83.(D). Skin biopsy is recommended for urticarial lesions that persist at the same
location for >24 hr, those with pigmented or purpuric components, and those that burn
more than itch. Collagen vascular diseases such as systemic lupus may manifest
urticarial vasculitis as a presenting feature.
84(E).
85(E).
86(8). Vaccination is a trigger, not a cofactor in anaphylaxis, others are cofactors.
87.(A). Principal pathologic features in fatal anaphylaxis include acute bronchial
obstruction with pulmonary hyperinflation, pulmonary edema, intraalveolar
hemorrhaging, visceral congestion, laryngeal edema, and urticaria and angioedema.
Acute hypotension is attributed to vasomotor dilation and/or cardiac dysrhythmias.
88(3). Other choices are to be considered in cases where there are no cutaneous
manifestations.
89(C).
90.(D). Plasma histamine is elevated for a brief period but is unstable and difficult to
measure in a clinical setting. Plasma tryptase is more stable and remains elevated for
several hours but often is not elevated, especially in food-induced anaphylactic
reactions.
91(3). Patients may experience biphasic anaphylaxis, which occurs when anaphylactic
symptoms recur after apparent resolution. More than 90% of biphasic responses occur
within 4 hours, so patients should be observed for at least 4 hr before being discharged
from the emergency department.
92.(A).
93(A).
94(3). Symptomslasting longer suggest another diagnosis.
95(8). Thrombocytopeniais often present.
96(0). Not specificand not diagnostic.
222
97.(E).
98(3).
99.(A). Heiner syndrome (food induced pulmonary hemosiderosis) is a nonlgE
mediated food allergy.
100.(C).The onsetis usually during adulthood. Othersare earlier ln presentation.
101(0). Commonly provoked by ingestion of milk and soy based protein formula.
102.(C).
103.(A).
104(8).
105(0).
106.(C). There are no laboratory studies to help identify foods responsible for cell-
mediated reactions. Consequently, elimination diets followed by food challenges are
the only way to establish the diagnosis.
107.(E).
108.(C). Antibacterial sulfonamldes, anticonvulsants, oxicam NSAle, and allopurinol
have been implicated in causing Stevens - Johnson syndrome and toxic epidermal
necrolysis.
109(8). Hydrochlorothiazide and calcium channel blockers can cause cutaneous lupus.
110.(C). Tetracycline, NSAIOs, and carbamazepine are implicated in causing nxed drug
eruption.
111.(D). Nitrofurantoin, bleomycin and methotrexate are implicated ln causing
pulmonary hbrosis.
112.(C). They can cause anaphylaxis.
113.(A). Along with Hydralazine and procainamide.
114.(C). Along with Hydralazine and propylthiouracil.
115.(E). Atopy does not appear to predispose patients to allergic reactions to low-
molecularweight compounds, but atopic patients in whom an allergic reaction
develops have a significantly increased risk of serious reaction.
116.(C).
223
RheumaticDiseasesof Childhood
QUESTIONS
USAMA AUUMAILY
1. Nonsteroidal drugs
anti-inflammatory (NSAIDs) to decrease
areprescribed acuteand
chronicinflammation with variousrheumaticdiseases;however,many
associated
adverse effects have been encountered with long term use.
Of the following,the NSAIDthat hasthe highesttoxicityis
A. celecoxib
B. meloxicam
C. indomethacin
D. naproxen
E. ibuprofen
3. Hydroxychloroquine
sulfateisan antimalarial
drugimportantin the treatmentof SLE
anddermatomyositis, cutaneous
particularly of diseaseand to reduce
manifestations
lupus flares.
of the following,the MOST
importantProcedurethat should be done routinely during
the courseof administrationis
A. gastricendoscopy
B. bone marrow examination
C. musclebiopsy
D. glucose-G-phophate
E.
dehydrogenase levelassay
enzyme
Ophthalmological
examination
224
C. systemic llA
D. peripheralarthritisassociatedwith juvenile ankylosingspondylltis
E. enthesitis associated with juvenile ankylosing spondylitis
6. Juvenile idiopathic arthritis has many subtypes. Arthritis must be present to make a
diagnosis of any subtype; involved joints oftenly have the following signs EXCEPT
A. swelling
8. warm
C. erythema
D. limitation of movement
E. pain on movement
8. Oligoarthritis predominantly affects the joints of the lower extremities rather than
upper extremity joints. Which of the following joints is never a presenting sign of
oligoarthritis?
A. hip
B. knee
C. ankle
D. metatarsal
E. interphalangeal
225
9. A 4-year-old girl recently diagnosed with persistent oligoarticular juvenile idiopathic
rheumatoid arthritis (JlA); she has 3 involved )oints including the right knee, right ankle,
and left elbow; antinuclear antigen (ANA) is significantly positive.
Of the following, the MOST important step in the management of this girl is
regular examination of locomotor system
periodic slit-lamp examination
.MPDSD?
periodic ANA monitoring
periodic erythrocyte sedimentation rate (ESR) monitoring
frequent C-reactive protein (CRP) monitoring
All the following are more likely to be correlated with ANA positivity EXCEPT
anterior uveitis
e
9.00."?
female sex
symmetrical arthritis
lower number of involved joints over time
12. A 5-year~old boy develops acute onset of high spiking fevers, lymphadenopathy,
hepatosplenomegaly, and purpura; he has been diagnosed with systemic Juvenile
idiopathic arthritis (JlA) since early childhood treated with antiinflammatory drugs. You
suspectmacrophage activating syndrome (MAS).
Of the following, the BESTtest that distinguishesMAS from a flare of the primary
disease is
A. leucopenia
B. failing ESR
C. hypofibrlnogenemia
D. hypertriglyceridemia
E. evidence of hemophagocytosls in the bone marrow
13. Rheumatoid factor (RH-positive polyarthritis usually accounts for < 10% of all
juvenile idiopathic arthritis (JlA) cases. The articular manifestation pattern is
characterized by involvement of 25 joints in both upper and lower extremities.
of the following, the LEASTeffective drug to Induce remission for this subtype of MA is
A. NSAiDs
B. methotrexate
C. TNF-u antagonists
D. IL-1 inhibitors
E. lL-6 inhibitors
14. A 5-year-old boy has systemic juvenile idiopathic arthritis (leA) with systemic
manifestations including fever, hepatosplenomegaly, lymphadenopathy, and
pericarditis.
Of the following, the BESTinitial treatment for this boy is
A. NSAIDs
B. systemic glucocorticoids
C. TNF-a inhibitors
D. iL~1 antagonists
E. .6 antagonists
15. You are discussing with the medical students the role of systemic steroids for the
management of rheumatologic diseases.
Your discussion should include all the following statements EXCEPT
A. they are recommended for management of severe systemic illness
B. they are used for bridge therapy during the wait for therapeutic response to a
disease modified anti rheumatic drugs (DMARD)
C. they are effective for control of uveitis
D. they prevent joint destruction
E they impose risks of severe toxicities
17. The child with polyarticular .llA often has a more prolonged course of active joint
inflammation and requires early and aggressive therapy.
0f the following, the predictor that carries the WORST prognosis is
227
old age at onset
rheumatoid factor (RF) seronegativity
FPPP?enthesitis
asymptomatic
gastrointestinal
uveitis
inflammation
19. Enthesitis-Related Arthritis (ERA)is a subtype of rheumatic disease that falls in the
juvenile idiopathic arthritis (JIA) category.
0f the following, the clinical feature that is highly suggestive of ERA is
A. symmetrical arthritis early in the disease course
8. early involvement of more than 5 joints
C. involvementof upper limb joints
D. Inflammation of the small joints of the foot
E. asymmetrical enthesitis
21. You are discussing with medical students Juvenile Ankylosing Spondylltis (JAS)
subtype of spondyloarthritides. One of the students asks you about the features that
distinguish this disease from the adult-onset ankylosing spondylitis (AOAS).
All the following are true regarding JAS in comparison with AOAS EXCEPT
it is present in patients <16 yr old
axial disease occurs more frequently early in the dlsease course
228
22. Reactive arthritis is defined as joint inflammation caused by a sterile inflammatory
reaction following a recent infection.
Of the following, the LEASTlikely micro-organismthat may cause reactive arthritis is
A. Clostridium difficile
B. Salmonella paratyphi
C. Shigella flexneri
D. Yersinia enterocolitica
E. Campylobacterjejuni
23. The distinction between postinfectious arthritis and reactive arthritis is not always
clear. All the following are common features of postinfectious arthritis, rather than
reactive arthritis EXCEPT
A. there is a transient joint swelling or pain
B. it lasts less than 6 wk
C. chronic spondyloarthritis is a common sequelae
D. it may be encountered after group A streptococcus infection
E. rubella infection is a common causative agent
24. You are meeting parents of 2-year-old girl who is going to receive rubella vaccine;
the mother is concerned regarding arthritis that may develop after immunization.
Of the following, the statement that should be included during the discussion is
it is usually frequently encountered after immunization
it typically affects the large joints of the upper extremity
25. Postinfectious arthritis describes arthritis that occurs after infectious illnesses,
mainly viruses.
Of the following, the LEASTlikely causative viral infection is
A. rubella
varicellazoster
FPO!
cytomegalovirus
Epstein-Barr virus
herpes-simplex virus
26. A 4-year~old boy has an acute severe left groin pain that refers to thigh.
Examination reveals limitation of movement of the left hip joint. Lab investigations
reveal normal blood count and indices, and normal ESR; radiologic imaging shows
widening of the hipjolnt space with effusion. You suspect transient synovitis.
0f the following, the BESTtherapeutic approach for this boy is
A. systemic steroids
229
methotrexate
aspirationof joint fluid
W90? intravenous
observation
antibiotics
A Escherichia coli
B. Chlamydia trachomatis
C. Proteus mirabilis
D Pseudomonas aeruginosa
E Staphylococcus saprophyticus
29. Skin is a commonly involved organ by SLE. There are different cutaneous
manifestations.
0f the following, the skin manifestation that is MOSTsuggestiveof SLEin children is
malar rash
discoid rash
91.0.09"?
photosensitive rash
cutaneous
livedo
vasculitis
reticularis
230
D. penicillin
E. tetracycline
31. Systemic
lupuserythematosus isoftencharacterized
(SLE) of flareand
byperlods
diseasequiescenceor may follow a more smolderingdiseasecourse.
All the following lab tests correlate with active disease EXCEPT
A positive anti-nuclear antibody titer
B. positive anti-double-stranded DNA level
C. low serum complement level
D. high erythrocyte sedimentation rate
E. elevated C-reactive protein (CRP) value
32. A 12-year-old female adolescent has a recent diagnosis of SLE;she has a faint malar
rash and mild arthritis including both elbow and knee joints as well as the joints of
.KUK
small fingers.
Of the following, the MOST appropriate initial therapy for this patient is
A systemic steroids
B. methotrexate
C. leflunomide
D. cyclophosphamide
E. hydroxychloroquine
33. A 3-week-old male baby develops malar rash involving the face and periorbital area
after exposure to sun light; you suspect neonatal lupus.
0f the following, the NEXT step of management is
A. measurement of anti-Ro and anti-La antibodies of the mother
B. measurement of anti-Ro and anti-La antibodies of the baby
C. cardiac evaluation of the mother
D. cardiac evaluation of the baby
E hematological evaluation of the baby
34. You are meeting a pregnantmother who has had a previousbaby with congenital
heart block due to neonatal lupus;she has a positiveanti-SSAand anti-SSBantibodies.
All the following are therapeutic modalitiesto prevent occurrenceof congenitalfeta|
cardiac complications EXCEPT
A. fluorinated corticosteroids
B. intravenous immunoglobulin
C. plasmapheresis
D hydroxychloroquine
E methotrexate
231
35. Juvenile
dermatomyositis (JDM)is the most commoninflammatorymyositisin
by skinrashandproximalmuscleweakness.
childrencharacterized
All the followingare commoncutaneousmanifestationsof JDM EXCEPT
A. heliotrope rash of the eyelids
B. photosensitivity to ultraviolet light
C. facial erythema sparing the nasolabial folds
D. Gottron papules
E. periungualtelangiectasias
FLUFF?
avoidance of sun exposure
bed rest
social and psychological
38. Juvenile localized scleroderma (11.5)is generally insidious with skin manifestations
that vary according to disease subtype. Up to 25% of children with LS have
extracutaneous manifestations, including arthritis and neurological symptoms.
Of the following, the subtype that is MOST commonly associated with neurological
manifestations is
A. plaque morphea
eosinophilic fasciitis
W909
generalized morphea
en coup de sabre
morphea profunda
232
of
J S) has a Prolonsedcoursewith periods
'
39. Juvenlie sys I roderma
temlcsce (S
remissionand exacerbation.It is characterizedby multisystemorgan
involvement
including skin, Iocomotor system, CNS, and viscera.
0f thefollowing,
theMOST
common ls
manifestation
earlycutaneous
edema of the dorsum of the hands and fingers
induration and fibrosis of the skin
91539.07?
flexion contracturesat the elbows, hips, and knees
skin ulceration over pressure points
subcutaneous
40.Youarediscussing
calcifications
phenomenon
Raynaud (RD)with
disease
(RP)andRaynaud
medical
students. associated
YoustatethatRPisusually while
diseases,
withrheumatic
RD is independent of an underlying rheumatic disease.
with RD,ratherthan RPis
thefeaturethat isMOSTlikelyconsistent
Ofthe following,
presence in early childhood
absence of tissue necrosis and gangrene
0.005?
asymmetric occurrence
presence of periungual telangiectasia
associated pain and paresthesia
43.Primary
Sjogren
syndrome, rare,mayoccur
although inchildren
between
9~10
year
233
common
theMOST
Ofthefollowing, Is
Inchildren
manifestation
A. recurrent parotitis
B. sicca symptoms
C. polyarthrltis
D. vulvovaginitls
E. hepatitis
fever(FMF)Is a recessively
Mediterranean
44. Familial autoinflammatory
inherited
disease
usually by recurrent
characterized self-limited
episodesof fever,serositis,
arthritis, and skin rash.
findingof thisdiseaseis
Of the following,the hallmarkcutaneous
A. erysipelas-llkeerythemaoverlyingthe dorsumof the foot
B. morbiiliform rash
C. migratory rashoverlyingarea of myalgia
D. cold-induced urticaria like lesions
E. generalized pustulosls
of familialMediterraneanfever (FMF),
isthe mostseriouscomplication
45. Amyioidosis
and in its absence FMF patients may live a normal life span.
Of the following, the organ that is not affected by secondaryamyloidosisof FMF is
A. kidney
8. lung
C. nerve
D. heart
E. teste
47. Although
thereis no established
therapyto AAamyloidosis,
colchicine
maybe an
effectivedrugto preventthe developmentof amyloidosis.
thefollowing,
thedisease
thatisrespond
tocolchicine
inpreventing
AAamyloidosis
:3f
s
234
ankylosing spondylitis
W90?
familial Mediterranean fever
hyper lgD syndrome
cryopyrin-associated periodic syndrome
49. Kawasaki disease (KD) has unique clinical manifestations; however, less consistent
clinical presentation may occur.
All the following are common classic clinical features of KD EXCEPT
A. bilateral non exudative bulbar conjunctivitis
8. cracked lips
C. edema and erythema of the hands and feet
D. vesicular rash
E. unilateral non suppurative cervical lymphadenopathy
50. You are evaluating a 1-year-old boy with an established diagnosis of Kawasaki
disease (KD) since 4 days; the caring nurse asks you about cardiac involvement during
this stage.
All the following are possible cardiac complications at this stage EXCEPT
A. myocarditis
B. pericarditis
C. coronary artery aneurysm
D. mitral regurgitation
E. cardiogenic shock
is performedto a 1.5~year-oldboy
51. A follow up two-dimensionalechocardiography
with Kawasakidisease(KD) 3 weeks after the diagnosis;it shows a small solitary
aneurysmof the left anterior descendingcoronary artery without thrombosis.
0f the following, the MOSTappropriate therapy for this child is
A. aspirin for 6 months
B. life-long aspirin
C. IVIG and aspirin for 14 days
D. aspirin and clopidogrel antiplatelet for 8 weeks
E. aspirin and warfarin for 6 months
52. Henoch-Schdnlelnpurpura (HSP) is the most common vascuiitis of childhood
affecting small vessels in the skin, lolnts, gastrointestinal tract, and kidney.
Gastrointestinal involvement: occur in up to 80% of children with HSP.
Of the following, the LEASTgastrointestinal manifestation that may occur in children
with HSP ls
. abdominal pain
vomiting and diarrhea
53. A 5-year-old boy has acute onset of abdominal pain and meiena; he has been
diagnosed with Henoch-Schonleln purpura (HSP) one week before. Examination reveals
symmetrical palpable purpura overlying the lower extremities and buttock; other
examinations are unremarkable.
Of the following, the BESTtreatment for this boy is
A. supportive measures
8. steroids
C. azathioprine
D. cyciophosphamide
E. mycophenolate mofetil
54. Renal disease is the major long-term complication, occurring in 1-2% of children
with Henoch-Schbnlein purpura (HSP). Chronic HSP renal disease is managed with a
variety of lmmunosuppressants.
Of the following, the LEASTeffective drug for the treatment of renal disease in HSP is
A. prednisolone
B. azathioprine
C. cyclophosphamide
D. cyclosporine
E. mycophenolate mofetil
1.(C). Certain agents (indomethacin) have a higher risk of toxicity than others
(ibuprofen); naproxen has an intermediate risk. Selective COX~2 inhibitors (such as
ceiecoxib and meloxicam) inhibit receptors responsible for promoting inflammation
with potential for fewer gastrointestinal adverse effects.
2.(D). Naproxen is more likely than other NSAIDs to cause pseudoporphyria.
Pseudoporphyria is more likely to occur in fair-skinned individuals and on sun-exposed
areas. lf pseudoporphyria develops; the inciting NSAID should be discontinued because
scars can persist for years or be permanent.
1(6). The most significant potential adverse effect is retinal toxicity, which occurs
rarely but results in irreversible color blindness or loss of central vision. Complete
ophthalmologic examinations, including assessment of peripheral vision and color
fields, are conducted at baseline and every 612 mo to screen for retinal toxicity.
4.(c). Sulfasalazine is generally considered contraindicated in children with active
systemic JIA because of increased hypersensitivity reactions.
S.(A). Nausea, vomiting, anorexia, alopecia, mucositis, hemorrhagic cystitis, and bone
marrow suppression are potential short-term adverse effects.
6.(C). Involved joints are often swollen, warm to touch, and painful on movement or
palpation with reduced range of motion, but usually are not erythematous.
7(8). ln oligoarthritis, it predominantly affects the large joints of the lower extremities,
such as the knees and ankles. isolated involvement of upper extremity large joints is
less common.
8.(A). isolated involvement of the hip is almost never a presenting sign and suggests
extended oligoarticularjuvenile idiopathic arthritis.
9.(B). The presence of a positive ANA confers increased risk for asymptomatic anterior
uveitis, requiring periodic slit-lamp examination.
10.(D). ANA positivity is more likely to be correlated with asymmetric arthritis.
11.(D). Rheumatoid nodules on the extensor surfacesof the elbows, spine, and over
the Achilles tendons, although unusual, are associated with a more severe course and
almost exclusively occur in RF-positive individuals.
12.(E). The most important indicators of MAS include a falling platelet count, extreme
hyperferritinemia, increased liver enzymes, falling leukocyte count, persistent,
continuousfever 238°C,falling ESR,hypofibrinogenemia,and hypertriglyceridemia.The
diagnosisis confirmed by bone marrow biopsy demonstrating hemophagocytosis.
13.(A). NSAle alone rarely induces remission in children with polyarthritis or systemic
JIA. Methotrexate is the oldest and least toxic effective drug. Biologic medications that
237
inhibit proinflammatory cytokines, such as TNF-a, lL-1, and lL-6, demonstrated
excellent disease control.
14.(B). TNF inhibition is not as effective for the systemic symptoms found in leA. When
systemic symptoms dominate systemic steroids are started followed by the initiation of
lL-1 or IL6 antagonist therapy, which often induces a dramatic and rapid response.
1540). Systemic steroids do not prevent joint destruction.
16.(E). There is no association between the activity or severity of arthritis and uveitis.
17.(E). Disease involving the hip and hand and wrist is associated with a poorer
prognosis and may lead to significant functional impairment. All other mentioned
factors carry a good outcome.
18.(D). Symptomatic, rather than asymptomatic, eye inflammation (acute anterior
uveitis) is a clinical feature suggestive of spondyloarthritis.
19.(D). In ERA, the arthritis is typically asymmetric and involves 4 or fewer joints during
the 1 6mo of the disease.The most frequently affected joints are the knees, ankles,
and hips. Enthesitis is typically symmetric and most commonly affects the lower limbs.
Inflammation of the small joints of the foot, or tarsitis, is highly suggestive of ERA.
20.(E). Positive rheumatoid factor is never associated with all subtypes of
spondyloarthritides.
21.(B). in comparison to adult-onset AS, axial disease and inflammatory back pain are
less frequent at disease onset, while enthesitis and peripheral arthritis is more
common.
22.(A). Reactive arthritis typically follows enteric infection with Salmonella sp., Shigella
flexneri, Yersinia enterocolitica, Campylobacter jejuni, orgenitourinary tract infection
with Chlamydia trachomatis. Escherichiacoli and Clostridium difficile are also causative
enteric agents, although less common.
23.(C). Postinfectious arthritis does not necessarily share the typical spondyloarthritis
pattern of joint involvement.
24.(D). Rubella-associated arthropathy may follow natural rubella infection and,
infrequently, rubella immunization. it typically occurs in young women, with an
increased frequency with advancing age, and is uncommon in preadolescent children
and in males. Arthralgia of the knees and hands usually begins within 7 days of onset of
the rash or 10-28 days after immunization.
25.(D).
26.(C). ln transient synovitis (toxic synovitis), aspiration of joint fluid is often necessary
to exclude septic arthritis and typically results in dramatic clinical improvement.
27.(B).
28.(C). Childhood SLEis rare before 5 yr of age.
29.(8).
30.(A).
31.(A). ANA titers are not reflective of disease activity; therefore, repeating ANA titers
is not helpful in disease management.
238
31(5). Hydroxychioroquine is recommended for all individuals with SLE if tolerated. In
addition to treating mild SLE manifestations such as rash and mild arthritis,
hydroxychloroquine prevents SLE flares, improves lipid profiles, and may have a
beneficial impact on mortality and renal outcomes. Corticosteroids are a mainstay for
treatment of significant manifestations of SLE. Methotrexate, lefiunomlde, and
azathioprine are often used to treat persistent moderate disease, Including arthritis,
signincant cutaneous or hematologic involvement, and pleural disease.
Cyclophosphamide is reserved for the most severe, potentially life threatening SLE
manifestations, such as renal, neurologic, and cardiopulmonary disease.
33.(D). The most serious complication due to neonatal lupus is congenital heart block.
34(5).
35.(C). in JDM, facial erythema is crossing the nasolabiai folds, in contrast to the malar
rash without nasoiabial involvement typical of systemic lupus erythematosus.
36.(D). Bed rest is not indicated, because weight bearing improves bone density and
prevents contractures.
37.(C). Cardiac involvement by JDM is rare.
38.10). Children with en coup de sabre may have symptoms unique to central nervous
system involvement, such as seizures, hemifaciai atrophy, ipsilaterai uveitis, and
learning/behavioral changes.
39.(A).
40(8). RD often begins in adolescence and is characterized by symmetric occurrence,
the absence of tissue necrosis and gangrene, and the lack of manifestations of an
underlying rheumatic disease. Children have normal naii~fo|d capillaries. Pain and
paresthesia indicate ischemic changes, associated only with RP due to rheumatic
diseases.
41.(D). The oral ulcers heal without scarring.ln contrast; the genital ulcers heal with
scars.
441A).
45.(C). Amyioidosis may develop most commonly in the kidneys, gastrointestinal tract,
Spleen, lungs, testes, thyroid, and adrenals. Rarely, cardiac amyloidosis may develop;
rnacrogiossia and amyloid neuropathy is generally not seen with the amyloidosis of
PMF.
239
46.(A). JIA is a rheumatic disease that is associated with the development of AA
amyloidosis with the highestprevalence in patients with systemic A followed by those
with polyarticulardisease.
47.(C). Unlike AA amyloidosisassociated with FMF, AA amyloidosis associated with
other autoinflammatory diseases (including TRAPS, cryopyrin-associated periodic
syndrome,and HlDS) and chronic rheumatic diseases (JIA, RA, and ankylosing
spondylitis)do not respondto colchicine.
48.(A). Youngage is poor predictor. Other lab findings associated with poor outcome
are neutrophilia, thrombocytopenia, transaminitis, hyponatremia, hypoalbuminemia,
elevatedlevelsof N-terminal-probrain natriuretic protein.
49.(D). Typicalrash is maculopapular,erythema multiforme, or scarlatiniform. Bullous,
pustular,or vesicularrashesare uncommon skin manifestations.
Coronary
SO.(C). arteryaneurysm in the subacutephasein the 2"'1or
usuallydevelops
3rdweekof the illness.
51.(B).The patient with KD who has had a small solitary aneurysm should continue
aspirin indefinitely. Patients with larger or numerous aneurysms may require the
additionof other antiplatelet agents or anticoagulation.
52.(E).lntussusception, mesenteric ischemia, and intestinal perforation are uncommon
in childrenwith HSP.
53.(B).Steroidis most often used to treat significant gastrointestinal involvement or
other life-threatening manifestations.
54.(A).
55(5).
240
- Infectious
Diseases
QUESTIONS
HAYDER ALM USAWI
2. The optimal amount of blood to collect from a pediatric patient for blood culture
depend on
A. age
B. weight
C. length
D. BMi
E. laboratory standards
3. The IgM response occurs earlier in the illness, generally peaking at 7-10 days after
infection, and usually disappears within a few weeks, but for some infections it can
persist for months such as
A. measles
B. mumps
C. rubella
D. hepatitisA
E. varicella
241
C. pneumococcal
D. varicella
E. diphtheria
vaccine
6.Rotavirus notbeinitiated
should than
older
forinfants
A. 11 wk
B. 13 wk
C. 15 wk
D. 17 wk
E. 19wk
no laterthan
7.Thefinaldoseof rotavirusvaccinemustbeadministered
A. 6 mo of age
B. 8 mo of age
C. 10 mo of age
0. 12 mo of age
E. 18 mo of age
.MPOPP measles
rubella
pertussis
wpne? 12 diseases
13 diseases
14 diseases
10.TheMOSTcommonadversereactionto intramuscular
immunoglobulinis
A. pain at the injection site
B. flushing
C. headache
D. chills
E. nausea
11.Allthefollowingaremajor
recommended
indications
for IVIGEXCEPT
242
replacement therapy for primary immunodeficiency disorders
Kawasaki disease
W909?
hepatitis A prophylaxis
immune-mediated
prophylaxis of
thrombocytopenia
infection following bone marrow transplantation
meow?
aseptic meningitis
carditis
renal insufficiency
13. A toxoid is a modified bacterial toxin that is made non-toxic but still able to induce
an active immune response against the toxin.
Which of the following vaccine is a toxoid?
hepatitis A
hepatitis B
wpnw? pneumococcal
varicella
diphtheria
14. Hepatitis A vaccine, licensed for administration to children 12 mo of age and older.
The 2 doses in the series should be separated by at least
A. 2 mo
3. 4 mo
C. 6 mo
D. 1 yr
E. 2 yr
16. The minimum age for the last dose of hepatitis B vaccine is
A. 16 weeks
B. 20 weeks
C. 24 weeks
D. 28 weeks
243
E, 32 weeks
1600
weight
infant, should
gm, thefollowing
notreceive vaccine
atbirth
17Preterm
A. BCG
toaHBs
B,ifborn
B. hepatitis mother
Agnegative
C. polio
D. DPT
E. MMR
of thefollowingvaccines
18.Which for a patientwithX-Iinked
is contraindicated
agammaglobulinemia?
A. BCG
B. hepatitis B
C. DPT
D. MMR
E. varicella
iscontraindicated
of'thefollowingvaccines
19.Which renal
fora patientwithchronic
disease?
A. pneumococcal
B. hepatitis B
C. live attenuated influenza
D. varicella
E. hepatitis A
20. Waterless hand hygiene products are effective in killing most microbes but do not
remove dirt or debris and are ineffective against
A. Pseudomonas
B. hepatitisA
C. Salmonella
D. C. difficile
E. S. aureus
21. All the following are recognized skin infections or infestations in children in
childcare EXCEPT
A. impetigo
B. pediculosis
C. scabies
D. erythrasma
E. tinea corporis
22.A 3-year-old
childdiagnosed
withshigella
infection,he canreturnto hisdaycare
244
2 days after initiation of treatment
23. Oral rehydration is the mainstay of treatment for pediatric traveleis diarrhea and
the drug of choice is
metronidazol
azithromycin
F1905?
amoxicillin
trimethoprim-sulfamethoxazol
erythromycin
25. Three mechanisms can produce fever: pyrogens, heat production exceeding loss,
and defective heat loss. Endogenous pyrogens include
A. antigen-antibody complexes
B. complement components
C. lymphocyte products
D. interferons B and v
E. androgenic steroid metabolites
245
C. malaria
D. trichinosis
E. toxoplasmosis
in a childwith FUOsuggests
28. Bulbarconjunctivitis
A. leptospirosis
B. coxsackievirus infection
C. tuberculosis
D. infectious mononucleosis
E. lymphogranuloma venereum
mayberequiredto detectbacteremia
29. Multiplebloodcultures with
associated
A. malaria
B. pyelonephritis
C. brucellosis
D. osteomyelitis
E. pneumonia
30. Primary immunodeficiencies are compromised states that result from genetic
defects affecting 1 or more arms of the immune system while secondary
immunodefnciencies result from infection, malignancy, or as an adverse effect of
immunomodulating or immunosuppressing medications.
Which of the following represent secondary immune deficiency?
A. Shwachman-Diamond syndrome
8. cysticfibrosis
C. ChédiakHigashi syndrome
D. Omenn syndrome
E. ataxia-telangiectasia
31.Whichtypeof feverispersistent
andvariesby morethan0.S°c(O.9°f)/day?
A intermittent
B. hectic
C. sustained
D. remittent
E. relapsing
91.0.0.0?
infection
drug fever
heat stroke
246
33. Relative bradycardia(when the pulserate remainslow in the presenceof fever) can
accompany all the following conditions EXCEPT
A. typhoid fever
3. brucellosis
C. leptospirosis
D. visceral leishmaniasis
E. drug fever
34. The MOST common serious bacterial infection in infant aged 1-3 mo is
A. pyelonephritis
B. meningitis
C. pneumonia
D. septic arthritis
E. osteomylitis
36. Leukocyteadhesion defects are causedby defects in the 8 chain of integrin (C018),
which is required for the normal processof neutrophil aggregation and attachment to
endothelial surfaces.
It is characterized by all the following EXCEPT
delayed cord separation
recurrent infections
38-Cefixime
isa third-generation active38'3Stallthe70"0Wl08
cephalosporln E)(CEPT
A. Streptococci
247
B. Staphylococci
C. H. influenza
gonorrhoeae
D. Neisseria
E. Proteusvulgaris
active
isa cephalosporin
39 Cephalexin S.aureus,
against E.coli,
Streptococcus,
Towhich
andProteus.
Klebsiella, of
generation it
cephalosporins
belongs?
A. 1
B. 2"d
3
3'3
mph 4t
5
t 3'
42. Cephalosporins
are widely used in pediatric practice, both in oral and parenteral
formulations.
Whichof the followingis a 2"dgenerationcephalosporins?
A. cefazolin
3- CePhalexin
C- Cefuroxime
0- Ceftazidime
5- CEftaroiine
43. Which
should not be mixed or reconstituted with a calcium-
cephalosporin
containi
such
35
A ngprogua
-
solution
Ringer orparenteral
'
nutrition
.
calcnum.
containing . i 7 .
320 in
3- cefotaxime
248
C. ceftriaxone
D. ceftazidime
E. cefepime
45. Which of the following represent a major criterion for the diagnosis of
staphylococcal toxic shock syndrome?
A. rash
B. conjunctivalhyperemia
C. myalgia
D. thrombocytopenia
E. vomiting
46. Kawasaki disease closely resembles toxic shock syndrome clinically. However, many
of the clinical features of toxic shock syndrome are usually absent or rare in Kawasaki
disease like
A. fever unresponsive to antibiotics
B. hyperemia of mucous membranes
C. erythematous rash
0. desquamation
E. diffuse myalgia
249
1
6
91.0.05?
12
18
29
49. Scarlet fever is an upper respiratory tract infection associated with a characteristic
rash.
Of the following, which statement is TRUE?
A. it is caused by an infection with pyrogenic endotoxin producing group A
streptococcus
8. the rash appears 96 hr after onset of symptoms
C. the rash begins to fade after 12 weeks
D before desquamation, the reddened papillae are prominent, giving the tongue
a strawberry appearance
E. the milder form can be confused with Kawasaki disease
50. impetigo (or pyoderma) has traditionally been classified into 2 clinical forms:
bullous and nonbullous.
Of the following, which statement is TRUE?
A. bullous impetigo is more common
8. nonbullous lesions are most common on the trunk and perineum
C. regional lymphadenitis is commonly associated with nonbullous lesions
0. nonbullous impetigo is generally accompanied by fever
E. bullous impetigo usually involve the face and extremeties
51. in which of the following circumstances the diagnosis of acute rheumatic fever can
be made without strict adherence to Jones criteria?
A. when chorea occurs as the only major manifestation of acute rheumatic fever
8. when indolent carditis is the only manifestation months after the apparent
onset of acute rheumatic fever
C. in a limited number of patients with recurrences of acute rheumatic fever in
particularly high-risk populations
D. all of the above
E. none of the above
250
F typically involves small joints
the pain can precede and can appear to be
0
disproportionateto the ablative
findings
D. rheumaticarthritis is almostdeforming
f" there is often a proportionalrelationshipbetween the severityof arthritis and
the severity of cardiac involvement
54. Patients with acute rheumatic carditis and more than minimal cardiomegaly should
receive prednisone 2 mg/kg/day in 4 divided doses for
A. 710 days
B. 2-3 weeks
C. 6-8 weeks
D. 2-3 months
E. 4-6 months
feverwith carditisbut
for peoplewho havehadacuterheumatic
57. Prophylaxis
Withoutresidualheart diseasepersistfor
islonger
A- 5 yroruntil21yrofage,whichever
islonger
3- 10yroruntil21yrofage,whichever
islonger
C- 5yroruntil40yrofage,whichever
251
islonger
D. 10yroruntil40yrofage,whichever
E. Lifelong
Bstreptococcus
58.Group orStreptococcus
(685), agalactiae, cause
isa major of
neonatalbacterialsepsis. . .
Ofthefollowing, common
theMOST syndrome GBS
withchildhood
associated disease
beyond early infancy is
bacteremia without a focus
meningitis
90?? ventriculitis
septic arthritis
pneumonia
common siteInvolved
secondary incases
ofpulmonary is
nocardiosis
62.1he
:95!
. rain
8. skin
C. kidney
252
D. liver
E. bone
purpura,necroticskinlesionsare lesscommonamongchildren
66. in meningococcal
treated with
A. penicillin G
B. ampicillin
C. cefotaxlme
D. ceftriaxone
E. meropenem
57.TheMOST
common severe
ofacute meningococcal is
septicemia
complication
A. arthritis
8. focal skin infarction
C. endocarditis
D. pneumonia
E. peritonitis
is
onpresentation
disease
factorfor invasivemeningococcal
58-A poorprognostic
253
A. hypertension
B. leukocytosis
C. alkalosis
D. meningitis . .
rate
sedimentation
erythrocyte
E normal
effective
theMOST forprophylaxis
agent of meningococcal
590fthefollowing,
disease is
A. ceftriaxone
B. rifampin
C. ampicillin
D. penicillin
E. amoxicillin
71. All patients who are presumed or proven to have gonorrhea should be evaluated
for concurrentpresenceof all the following infections EXCEPT
A. syphilis
B. hepatitis B
C. HlV
D. C. trachomatis
E. HSVZ
72.Children
whohavebacteremia
or arthritiscausedby gonococcalinfectionsshould
With
betreated ceftriaxone
(50mg/kg/day;
maximum: <45
if weighs
1g/day kg)fora
minimum
of
A. 3days
B. Sdays
C. 7days
D. 14days
E. 21days
73. Th6MOST -
common ofjointandboneinfections
etiology 5
inyoungChl'dren
\
254
A. Staphylococcus aureus
B. Streptococcus pneumoniae
C. Kingellakingae
D. Haemophilus influenza
E. Neisseria meningitidis
74. Diagnosisof chancroid in infants and children is a strong evidenceof sexual abuse.
Chancroid is caused by
A. Haemophilus ducreyi
B. Syphilis
C. C. trachomatis
D. Kingellakingae
E. Neisseria gonorrhea
76. The MOST common reason for which children receive antibiotics is
A. pneumonia
8. bronchitis
C. otitis media
D. pharyngitis
E. cystitis
255
beforea decisionismadebetweenhospital
mustbewitnessed
B. a paroxysm and
home care
C. suctioningof nose, oropharynx,or trachea should be performedon a
preventive schedule
than after
maybe moresuccessful
D. feedingin the periodfollowinga paroxysm
napping
E. familyeducation,recruitmentas partof the team, and continuedsupportafter
discharge are essential
79. Hospital dischargeof infants with pertussisis appropriate in all the following
circumstances EXCEPT
A. over a 24hr period diseaseseverity is unchangedor diminished
B intervention is not required during paroxysms
C. nutrition is adequate
D no complication has occurred
E. parents are adequately prepared for care at home
256
day of the illness
C. if complications not occur, the symptoms and physical findings gradually resolve
within 2-4 wk
D. typhoid fever usually manifests as high-grade fever with a wide variety of
associated features
E. relative bradycardia, neurologic manifestations, and gastrointestinal bleeding,
are rare in children
86. Cholera is a dehydrating diarrheal disease caused by Vibrio cholerae, of more than
200serogroups; the serogroups that have been associatedwith epidemics are
0131
0139
91.0.05?
0151
0159
0165
257
D. hypoglycemia
E. fever
91. The genus Yersinia is a member of the family Enterobacteriaceae and comprises
more than 14 named species, 3 of which are established as human pathogens.
Which of the following is MOST often associated with mesenteric lymphadenitis?
A. Yersinia enterocolitica
B. Yersinia pseudotuberculosis
C. Yersinia pestis
D. Yersinia moliaretii
E. Yersinia rohdei
92. Patients with conditions leading to iron overload are at higher risk of developing
infections with
A. Aeromonas
B. Pseudomonas aeruginosa
C. Yersinia
D. Francisella tularensis
E. Campylobacter
A. reactive arthritis
B. erythema multiforme
C. hemolyticanemia
D. thrombocytopenia
258
E. septicemia
W90?
oropharyngeal
oculoglandular
typhoidal
95. Human brucellosis is caused by organisms of the genus Brucella and continues to be
a major public health problem worldwide.
All the following are true EXCEPT
symptoms can be acute or insidious
most patients present with fever, arthralgia/arthritis, and hepatosplenomegaly
96.
opp?» some present as a fever of unknown origin
variable fever pattern
invasion of the nervous system occurs in approximately 20% of cases
909°?E
cephalosporin
macrolide
aminoglycoside
trimethoprim-sulfamethoxazole
97.Tetanusis an acute spastic paralytic illness historically called lock-jaw that is caused
by the neurotoxin produced by Clostridium tetani.
0f the following, the TRUE statement is
A. tetanus is most often localized
3. incubationperiodtypicallyis 2-6weeks
C- patientremainsconscious andthereisnopain[h
0- tetanicparalysis
becomes moresevereinthe4 wkafteronset
E. cephalic withchronicotitismedia
tetanusoccursin association
treatmentis caused
of acnein adolescentfemaleon anti-tuberculous
98.Worsening
W
isoniazid
PPS? rifampin
pyrazinamide
ethambutol
259
E. streptomycin
100. The ideal agent for treating fungal urinary tract infections is
A. amphotericin B
B. fluconazole
C. voriconazole
D. micafungin
E. caspofungin
101. Candida is a common cause of oral mucous membrane infections (thrush) and
perineal skin infections (Candida diaper dermatitis) in young infants.
All the following are true EXCEPT
A. candida species are the third most common cause of bloodstream infection in
premature infants
8. up to 10% of full-term infants are colonized as the result of vertical
transmission from the mother at birth
C. histamine-2 blockers facilitate Candida colonization and overgrowth
D. significant risk factors for neonatal invasive candidiasis include the presence of
a central venous catheter
E. the cumulative incidence is <0.3% among infants <750 g birthweight admitted
to the NICU
102. NICUs with a high incidence of invasive candidiasis should consider prophylaxis
with fluconazole in infants with a birthweight of
A. <750g
B. <1,000g
C. <1,500g
D. <2,000g
E. <2,SOOg
mph?» 9 mo
12 mo
18 mo
105. Acyclovir is a safe and effective therapy for herpes simplex virus (HSV) infections.
All the following are true EXCEPT
A. activity against CMV is less pronounced
B. activity against Epstein-barr virus is modest, both in vitro and clinically
C. acyclovir therapy in a nursing mother is not a contraindication to breastfeeding
D. main route of elimination is hepatic
E. high doses of acyclovir are associated with neurotoxicity
106. Ribavirin is a guanosine analog that has broadspectrum activity against a variety
of viruses, particularly RNA viruses.
All the following are true EXCEPT
A. its precisemechanismof action is incompletelyunderstood
8. aerosolized ribavirin effective for parainfluenza, influenza, and measles
infections
C. ribavirinis generallynontoxic,particularlywhenadministered
byaerosol
D. ribavirin and its metabolites concentratein hepatocytes
havebeenreportedfollowingexposureto
E. conjunctivitisand bronchospasm
aerosolized drug
W909?otitis
pneumonia
cystitis
media
111. The MOST common finding among infants with congenital rubella syndrome is
psychomotor retardation
cataracts
W905?deafness
patent ductus arteriosus
neonatal purpura
@905
pale opening of the Stensen duct
parotid swelling peaks in approximately 7 days
a morbilliform rash is rarely seen
113.TheMOSTcommoncomplicationof
mumpsis
A. meningitis
262
B. conjunctivitis
C. optic neuritis
D. pneumonia
E. thrombocytopenia
[9093
pulmonary stenosis
renal agenesis
no fetal malformations
115. Mumps virus is neurotropic and is thought to enter the CNS via the choroid plexus
and infect the choroidal epithelium and ependymal cells. CNS involvement is
characterized by
A symptomatic CNS involvement occurs in 40-60% of infected individuals
8. encephalitis most commonly manifests 10 days after the parotitis
C. CNS symptoms usually resolve in 3 days
D. CSFprotein content is usually elevated
E. facial palsy is a less-common CNS complication
is
menineitis
118.TheMOSTcommoncauseof recurrentaseptic
A- mumps
3- EBV
C. adenoviruses
D. HSV
E« influenzavirus
263
HSV
Inneonatal
119. encephalitis, inapproximately
occur
skinvesicles
20%
40%
mpow? 60%
80%
100%
121. Varicella-zoster virus (VZV) causes primary, latent, and recurrent infections.
All the following statements are true EXCEPT
A. varicella is a serious disease in young infants
B. within households, transmission of VZV occurs at a rate of 65-86%
C. Herpes zoster is more common in winter
D. the lifetime risk for herpes zoster for individuals with a history of varicella is 20-
30%
123. All the following are true about varicella in unvaccinated individuals EXCEPT
simultaneous presence of lesions in the same stages of evolution
distribution of the rash is predominantly central
264
All the following are true EXCEPT
A. morbidity from central nervous system complicationsis highest amongpatients
younger than 5 yr and older than 20 yr
8. nuchal rigidity, altered consciousness, and seizures characterize
meningoencephalitis
C. patients with cerebellar ataxia have a gradual onset of gait disturbance,
nystagmus, and slurred Speech
D. neurologic symptoms usually begin 2-6 days after the onset of the rash but may
occur during the incubation period or after resolution of the rash
E. clinical recovery is typically occurring after 96 hr, and is usually gradual
125. Oral therapy with acyclovir (20 mg/kg/dose; maximum: 800 mg/dose) given as 4
doses/day for 5 days can be used to treat uncomplicated varicella in individuals at
increased risk for moderate to severe varicella.
It includes all the following individuals EXCEPT
A. nonpregnant individuals older than 12 yr of age
8. individuals older than 12 mo of age with chronic cutaneous disorders
C. individuals receiving short-term corticosteroid therapy
D. individuals receiving long-term salicylate therapy
E. individuals with chronic hematological disorders
afteracuteinfectionfor
virus(EBV)is shedin oralsecretions
126.EpsteinBarr
A. 2 wk
B. 4 wk
C. 2 mo
D. 4 mo
E. 6 mo
127.
What infected
oftheworldspopulation virus(EBV)?
byEpstein-Barr
percent
A. 5%
B 25%
C 50%
D 75%
E 95%
128. clinical
isthebest-known caused
syndrome byEpstein-
Infectious
mononucleosis
Barrvirus (EBV),It is characterized by
A. symptomatic hepatitis
elevated liver enzymes
.rnpns»
massivesplenic enlargement
huge hepatomegaly
occasionalpalatal petechiae
265
The
129. common
MOST sequela
long-term CMV
withcongenital
associated infection
is
A. renalfailure
hearing loss
PS"
heartfailure
D. vision loss
E hepaticfailure
6 (HHV-6AandHHV-GB)
130.Humanherpesvirus 7 (Hi-NJ)
andhumanherpesvirus
HHV-GB
causeinfectionin infancyandearlychildhood. for the majorityof
isresponsible
casesof roseolainfantum (exanthema subitum or sixth disease).
All the following are true EXCEPT
A. 95% of children being infected with HHV-6 by 2 yr of age
B. peak age of primary HHV-6B infection is 6-9 mo of life
C. congenital infection with HHV-G occurs in 1% of newborns
D. congenital infection with HHV-7 is well recognized
E. breast milk does not play a role in transmission of either HHV-G or HHV-7
131. Amantadine and rimantadine are effective only against influenza A viruses and are
not approved for use in children younger than
6 mo
18 mo
W909?3 yr
5 yr
10 yr
132.Typically,
thefirstsignof infection
in infantswith respiratory virus(RSV)
syncytial
is
rhinorrhea
cough
mpnwelow-grade fever
increasedrespiratory rate
subcostal retractions
WPOF?3 and 6
4 and 7
5 and 8
134. Respiratorytract
infectionsare comm0"
(HAdV) . manifesta tions
ofhuman
«.00le
infections
inchIldren
andadults.
All the following are true EXCEPT
A. HAst cause 5-10% of all childhood respiratory disease
3, primary infections in infants may manifest as bronchiolitis or pneumonia
C. HAdV pneumonia may manifest as features more typical of bacterial disease
D. pharyngitistYPiCEIIIY inCIUdessymptomsof coryza,sore throat, and fever
E. HAdV can be Identified in <5% of children with isolated pharyngitis
138. amoebic
human
cancause
Allthefollowing EXCEPT
meningoencephalitis
A. Naegleria
B. Acanthamoeba
C. Balamuthia
D. Entamoeba histolytica
E. Sappinia
139.
One ofthefollowing statements Giardia
regarding isTRUE
lamblia
A. life cycle is composed of 3 stages . .
8- each 2 in
trophozontesthecaecum
ingested cystproduces
contain4 ovalnucleianteriorly
C. trophozoites
267
usedto
of chlorine
D. cystviabilityisnotaffectedbytheusualconcentrations
purifywaterfor drinking . . .
E. Giardia difference
genotypes of clinicalmanifestations
leadto widespectrum
141. Giardiasis should be considered in children who have the following presentations
EXCEPT
A. acute dysenteric diarrhea
B. persistent diarrhea
C. failure to thrive
D. malabsorption
E. chronic crampy abdominal pain
268
severe anemia
bleeding
W905?
secondary bacterial infection
hepatic failure
renal failure
269
Infectious Diseases
E ANSWERS
iiAYpERAiMUSAWI
1.(A).
2.(B).
3.(D). Hepatitis A and West Nile Virus.
4.(D). Vaccines can consist of whole inactivated microorganisms (e.g., polio and
hepatitis A), parts of the organism (e.g., acellular pertussis, HPV, and hepatitis B),
polysaccharide capsules (e.g., pneumococcal and meningococcal polysaccharide
vaccines), polysaccharide capsules conjugated to protein carriers (e.g., Hib,
pneumococcal, and meningococcal conjugate vaccines), live attenuated
microorganisms (measles, mumps, rubella, varicella, rotavirus, and live-attenuated
influenza vaccines), and toxoids (tetanus and diphtheria).
5.(C). T-lymphocyte independent vaccines are associated with poor immune responses
in children <2 yr of age, short~term immunity, and absence of an enhanced or booster
response on repeat exposure to the antigen. With some polysaccharide vaccines,
repeat doses actually are associated with reduced responses, as measured by antibody
concentrations, compared to lst doses (i.e., hyporesponsive). To overcome problems
of plain polysaccharide vaccines, polysaccharides have been conjugated, or covalently
linked, to protein carriers, converting the vaccine to a T-lymphocyte dependent
vaccine. In contrast to plain polysaccharide vaccines, conjugate vaccines induce higher-
avidity antibody, immunologic memory leading to booster responses on repeat
exposure to the antigen, long-term immunity, and herd protection by decreasing
carriage of the organism.
6.(C).
7.(B). Two rotavirus vaccines are available, RotaTeq (RVS) and Rotarix (RV1). With both
vaccines, the lst dose can be administered as early as 6 wk of age and must be
administered by 14 wk 6 days. The final dose in the series must be administered no
later than 8 mo of age. The RVS vaccine is administered in 3 doses at least 4 wk apart.
The RV1 vaccine is administered in 2 doses at least 4 wk apart. Immunization should
not be initiated for infants 15 wk of age and older as stated in the immunization
schedule.
8.(A). As a result of effective and safe vaccines,smallpox has been eradicated; polio is
close to worldwide eradication.
9.(B). Infants, children, and adolescentsin the Iraq routinely are immunized against 11
diseases: BCG, poliomyelitis, hepatitis B, diphtheria, tetanus, pertussis, H. influenzae
type b (Hib) disease, rotavirus, measles, mumps and rubella.
10.(A). The most commonadversereactionto immunoglobulinis pain and discomfort
at the injection site and, less commonly, flushing, headache, chills, and nausea.
270
11.(C).In hepatitis A prophylaxis,intramuscular lG is recommended.
12.(o).
13.(E).Toxoids are (tetanus and diphtheria).
14.(C).
15.(B).
Theminimumintervalbetween2"dand3rddoseis 8 weeksandatleast16weeks
16.(C).
after 1" dose. Minimum age for the last dose is 24 weeks.
17.(B). Preterm infants generally can be vaccinated at the same chronologic age as full-
term infants according to the recommended childhood immunization schedule. An
exception is the birth dose of hepatitis B vaccine. Infants weighing 22 kg and who are
stable may receive a birth dose. However, hepatitis B vaccination should be deferred in
infants weighing <2 kg at birth until 30 days of age, if born to a HBs Ag negative
mother. All preterm, low birth weight infants born to His Ag positive mothers should
receive hepatitis B immunoglobulin and hepatitis B vaccine within 12 hr of birth.
However, such infants should receive an additional 3 doses of vaccine starting at 30
days of age.
18.lA). Oral polio, smallpox, live attenuated influenza, and BCG all these vaccines are
contraindicated for this patient.
19.(C).
20.(D). Waterless hand hygiene products increase hand hygiene compliance and save
time; these agents are the preferred agents for routine hand hygiene when hands are
not visibly soiled. These products are effective in killing most microbes but do not
remove dirt or debris. However, they are ineffective against C. difficile spores, requiring
the use of other cleansing products during hospital C. difficile outbreaks.
21.(D).
22.(D).
23.(B). The drug of choice is azithromycin (10 mg/kg once daily for up to 3 days, with
maximum daily dose of 500 mg). Ciprofloxacin (10 mg/kg per dose twice a day for up to
3 days, maximum dose of 500 mg twice a day) is an alternative for children >1 yr of age.
Amoxicillin, trimethOprim-sulfamethoxazole (cotrimoxazole), and erythromycin should
not be prescribed for self-treatment of travelers diarrhea, because of widespread
resistance among diarrheal pathogens.
°
24.(C). Fever is defined as a rectal temperature 238 C (100.4°F) and a value >40°C
(104°F) is called hyperpyrexia.
25.(D). Endogenous pyrogens include the cytokines interleukins 1 and 6, tumor necrosis
factor a, and interferons B and y. Some substances produced within the body are not
pyrogensbut are capable of stimulating endogenous pyrogens. Suchsubstancesinclude
antigenantibody complexes in the presence of complement, complement
components, lymphocyte products, bile acids, and androgenic steroid metabolites.
26(3). Drugsthat are known to cause fever include vancomycin,amphotericin B, and
allopurinol.
271
27.(E).
Ingestion importantclueto infectionwith Toxocara
of dirt isa particularly canis
larvamigrans)
(visceral or Toxoplasma gondii(toxoplasmosis).
28.(A).Palpebralconjunctivitis in a febrile patient may be a clue to measles,
coxsackievirus infection, tuberculosis, infectious mononucleosis,
lymphogranulomavenereum,or cat-scratch disease. In contrast, bulbar conjunctivitis in
a child with FUOsuggests Kawasaki disease or leptospirosis.
29.(D). Multiple or repeated blood cultures may be required to detect bacteremia
associatedwith infective endocarditis, osteomyelitis, or deepseated abscesses.
30(8). All other distractors represent primary immune deficiency.
31.(D). Intermittent fever is an exaggerated circadian rhythm that includes a period of
normal temperatures on most days; extremely wide fluctuations may be termed septic
or hectic fever. Sustained fever is persistent and does not vary by more than 05°C
(0.9°F)/day. Remittent fever is persistent and varies by more than 0.S°C (0.9F)/day.
Relapsing fever is characterized by febrile periods that are separated by intervals of
normal temperature.
32.(C). Temperatures in excess of 41°C (105.8°F) are most often associated with a
noninfectious cause.
33.(D).
34.(A). Pyelonephritis is the most common serious bacterial infection in this age group
and is also more common in uncircumcised infant boys and infants with urinary tract
anomalies. E. coliis the most common pathogen identified in bacteremic infants, the
majority having pyelonephritis.
35.(A). Neutropenia is defined as an absolute neutrophil count of <1,000 cells/mm3
and can be associated with significant risk for developing severe bacterial and fungal
disease.
36.(D). Because the defect involves leukocyte migration and adherence, the neutrophil
count in the peripheral blood is usually extremely elevated but pus is not found at the
site of infection.
37.(B).Although there has been ever-increasingemergenceof resistanceto penicillins
but they remain the drugs of choice for pediatric infections caused by group A and
group B Streptococcus,Treponema pallidum (syphilis), L. monocytogenes, and N.
meningitidis.
38.(B).Activeagainststreptococci,H. influenzae,M. catarrhalis,Neisseriagonorrhoeae,
Serratia marcescens, and Proteus vulgaris. No antistaphylococcal or antipseudomonal
activity. Dose: 8 mg/kg/Z4 hr divided q 12-24 hr PO.
39.(A). Dose: 25-100 mg/kg/24 hr divided q 68 hr PO.
40.(B). Carbapenem antibiotic with broad-spectrum activity against Grampositive cocci
and Gram-negative bacilli, including P. aeruginosa and anaerobes. No activity against S.
maltophilia.
41.(D). It may increase levels of warfarin, phenytoin, and lithium.
42.(C). The first-generation cephalosporins (e.g., cefazolin, a parenteral formulation,
and cephalexin, an oral equivalent) are commonly used for management of skin and
272
soft-tissue infections caused by susceptible strains of S. aureus and group A
Streptococcus.The secondgeneration cephalosporins (e.g., cefuroxime, cefoxitin) have
better activity against Gram-negative bacterial infections than do first-generation
cephalosporins and are used to treat respiratory tract infections, urinary tract
infections, and skin and soft-tissue infections. A variety of orally administered second-
generation agents (cefaclor, cefprozil, loracarbef, cefpodoxime) are commonly used in
the outpatient management of sinopulmonary infections and otitis media. The third-
generation cephalosporins (cefotaxime, ceftriaxone, and ceftazidime) are typically used
for serious pediatric infections, including meningitis and sepsis. A fourth generation
cephalosporin, called cefepime, has activity against P. aeruginosa and retains good
activity against methicillin-susceptible staphylococcal infections. A fifth generation
cephalosporin, ceftaroline has been licensed.
43.(C). Ceftriaxone should not be mixed or reconstituted with a calcium-containing
product, because particulate formation can result. Cases of fatal reactions with
ceftriaxonecalcium precipitates in lungs and kidneys in neonates have been reported.
44.(C). Toxic shock syndrome (T55) is an acute and potentially severe illness
characterized by fever, hypotension, erythematous rash with subsequent
desquamation on the hands and feet, and multisystem involvement, including
vomiting, diarrhea, myalgias, nonfocal neurologic abnormalities, conjunctival
hyperemia, and strawberry tongue.
45.(A).
MAJOR CRITERIA (ALL REQUIRED)
1. Acute fever; temperature >38.8°C (101.8F)
2. Hypotension
3. Rash (erythroderma with convalescent desquamation)
MINOR CRITERIA (ANY 3 OR MORE)
1. Mucous membrane inflammation (vaginal, oropharyngeal or conjunctival
hyperemia, strawberry tongue)
2. Vomiting, diarrhea
3. Liver abnormalities (bilirubin or transaminase greater than twice upper limit of
normal)
4. Renal abnormalities (urea nitrogen or creatinine greater than twice upper limit
of normal, or greater than 5 white blood cells per high-power field)
5. Muscle abnormalities (myalgia or creatinine phosphokinase greater than twice
upper limit of normal)
6. Central nervous system abnormalities (alteration in consciousness without
focal neurologic signs)
7. Thrombocytopenia (100,000/mm3 or less)
46(6). Many of the clinical features of TSS are usually absent or rare in Kawasaki
disease,includingdiffuse myalgia, vomiting, abdominal pain, diarrhea, azotemia,
acuterespiratory
hVitiotension, distress andshock.
syndrome,
273
47.(A). Childrenat increasedrisk of pneumococcal infections include those with sickle
cell disease, asplenia, deficiencies in humoral (B cell) and complement-mediated
immunity, HIV infection, certain malignancies (e.g., leukemia, lymphoma), chronic
heart, lung, or renal disease (particularly nephrotic syndrome), cerebrospinal fluid leak,
and cochlear implants.
48.(C). A pharyngeal strains (e.g., M type 12) are associated with glomerulonephritis;
skin strains (e.g., M types 49, 55, 57, and 60) are considered nephritogenic. Several
pharyngeal serotypes (e.g., M types 1, 3, 5, 6, 18, 29), but no skin strains, are
associated with acute rheumatic fever.
49.(E). It is caused by an infection with pyrogenic exotoxin (erythrogenic toxin)-
producing GAS. The rash appears within 2448 hr after onset of symptoms, although it
may appear with the first signs of illness. After 3-4 days, the rash begins to fade and is
followed by desquamation. After desquamation, the reddened papillae are prominent,
giving the tongue 3 strawberry appearance. The milder form can be confused with viral
exanthems, Kawasaki disease, and drug eruptions.
50.(C). Nonbullous impetigo is the more common form. The lesions may occur
anywhere but are most common on the face and extremities. Regional lymphadenitis is
common. Nonbullous impetigo is generally not accompanied by fever or other systemic
signs or symptoms. Bullous impetigo is less common and occurs most often in neonates
and young infants. The usual distribution involves the face, buttocks, trunk, and
perineum.
51.(D).
52.(C). Arthritis occurs in approximately 75% of patients with acute rheumatic fever
and typically involves larger joints, particularly the knees, ankles, wrists, and elbows.
Involvement of the spine, small joints of the hands and feet, or hips is uncommon.
Rheumatic arthritis is almost never deforming. There is often an inverse relationship
between the severity of arthritis and the severity of cardiac involvement.
53.(A). Acute rheumatic carditis usually presents as tachycardia and cardiac murmurs,
with or without evidence of myocardial or pericardial involvement.
54.(B). Patients with carditis and more than minimal cardiomegaly and/or congestive
heart failure should receive corticosteroids. The usual dose of prednisone is 2
mg/kg/day in 4 divided doses for 2-3 wk followed by half the dose for 2-3 wk and then
tapering of the dose by S mg/24 hr every 2-3 days. When prednisone is being tapered,
aspirin should be started at 50 mg/kg/day in 4 divided doses for 6 wk to prevent
rebound of inflammation.
55.(C). Phenobarbital (16-32 mg every 6-8 hr PO) is the drug of choice. If phenobarbital
is ineffective, then haloperidol (0.01-0.03 mg/kg/24 hr divided bid P0) or
chlorpromazine (0.5 mg/kg every 46 hr PO) should be initiated. Some patients may
benefit from a few week courses of corticosteroids.
56(0).
57(3).
0 Rheumatic fever without carditis :5 yr or until 21 yr of age, whichever is longer
274
o Rheumaticfever with carditis but without residual heart disease : 10 yr or until
21 yr of age,whichever is longer
0 Rheumatic fever with carditis and residual heart disease: 10 yr or until 40 yr of
age, whichever is longer; sometimes lifelong prophylaxis
58.(A). Early-onset neonatal GBS disease presents as sepsis; pneumonia and meningitis.
Late-onset neonatal GBS disease most commonly manifests as bacteremia (45~65%)
and meningitis (25-35%). invasive GBS disease in children beyond early infancy is
uncommon. Bacteremia without a focus is the most common syndrome associated
with childhood GBS disease beyond early infancy.
59.(o).
o bacteremia without a focus 10 days
0 meningitis 2-3 wk
0 ventriculitis at least 4 wk
0 septic arthritis 3-4 wkase
o osteomyeiitis 34 wkas
60.(A). Specific antitoxin is the mainstay of therapy and should be administered on the
basis of clinical diagnosis. The role of antimicrobial therapy is to halt toxin production,
treat localized infection, and prevent transmission of the organism to contacts.
6118). Three major forms of actinomycosiscervicofacial, abdominal and pelvic, and
pulmonary. In the patient with cervicofacial actinomycosis, there is often a history of
oral trauma, oral surgery, dental procedures, or caries, facilitating entry of organisms
into cervicofacial tissues. Cervicofacial actinomycosis usually manifests as a painless,
slow-growing, hard mass and can produce cutaneous fistulas, a condition commonly
known as lumpy jaw. Less frequently, cervicofacial actinomycosis manifests clinically as
an acute pyogenic infection with a tender, fluctuant mass with trismus, firm swelling,
and fistulas with drainage containing the characteristic sulfur granules.
62.(A). The brain is the most common secondary site and is involved in 15-40% of cases
of pulmonary nocardiosis. Brain abscess is the most common presentation. and
meningitis is the second most common presentation.
63.(D). Actinomyces, staphylococcus, streptococcus, diphtheria, enterococcus, listeria
and nocardia are gram positive.
64.(B).
65.(A). The most common clinical manifestation of meningococcai infection is
asymptomatic carriage of the organism in the nasopharynx.
66(0).
61(8). The most common complication of acute severe meningococcal septicemia is
focal skin infarction, which most commonly affects the lower limbs and can lead to
Substantial scarring and require skin grafting.
88.03). Poor prognostic factors on presentation include hypothermia or extreme
hyperpyrexia, hypotension or shock, purpura fuiminans, seizures, leukopenia.
thrombocytopenia (including disseminated intravascuiar coagulation), acidosis, and
high circulatinglevels of endotoxin and tumor necrosisfactor-a. The presence of
275
petechiaefor <12 hr before admission,absence of meningitis, and low or normal
erythrocytesedimentation rate indicate rapid, fulminant progression and poorer
prognosis.
694A). Ceftriaxoneand ciprofloxacinare the most effective agents for prophylaxis, the
latter being the drug of choice in some countries. Rifampin is most widely used but fails
to eradicate colonization in 15% of mses. Neither penicillin nor ampicillin treatment
eradicates nasopharyngeal carriage and should not be routinely used for prophylaxis.
70.(E). Hematogenous dissemination occurs in 1-396 of all gonococml infections, more
frequently after asymptomatic primary infections than symptomatic infections. Women
account for the majority of cases, with symptoms beginning 7-30 days after infection
and within 7 days of menstruation. The most common manifestations are asymmetric
arthralgia, petechial or pustular acral skin lesions, tenosynovitis, suppurative arthritis,
and, rarely, arditis, meningitis, and osteomyelitis. Only 25% of patients complain of
skin lesions. .
71.(E). All patients who are presumed or proven to have gonorrhea should be
evaluated for concurrent syphilis, hepatitis 8, HIV, and C. tmchomau's infection.
72.(C). Children who have bacteremia or arthritis should be treated with ceftriaxone
(50 mg/kg/day; maximum: 1 g/day if weighs <45 kg) for a minimum of 7 days.
Meningitis should be treated for 10-14 days, and endowrditis for a minimum of 28
days, with ceftriaxone (50 mg/kg/ dose q12h with maximum of 1-2 g IV q12h). Neonatal
gonocowal ophthalmia is treated effectively with a single dose of ceftriaxone (50
mg/kg IM, not to exceed 125 mg); a single dose of cefotaxime (100 mg/kg IM) is an
acceptable alternative. The conjunctivae should be irrigated frequently with physiologic
saline solution.
73.(C). Kingellakingae is being increasingly recognized as the most common etiology of
joint and bone infections in young children.
741A). Chancroid is mused by Haemophilus ducreyi, a fastidious Gram negative
bacillus.
75.(C). The most important clinical manifestation of M.catarrhalis infection in children
is otitis media. Colonization and infection with M. catarrhalis are increasing in countries
in which pneumococal conjugate vaccinesare used widely.
76.(C). Otitis media is the most common reason for which children receive antibiotics.
on the basis of culture of middle ear liuid obtained by tympanocentesis, the
predominant causes of acute otitis media are Streptococcus pneumoniae, H.
influenzae,and M. catarrhalis.
77.(D). Typical paroxysmsthat are not life threatening have the following features:
duration <45 sec; red but not blue color change; tachycardia, bradycardia (not <60
beats/minin infants),or oxygendesaturationthat Spontaneouslyresolvesat the end of
the paroxysm; whooping or strength for brisk self-rescue at the end of the paroxysm;
self-expectorated mucus plug; and post-tussive exhaustion but not unresponsiveness.
78.(C). Suctioning of nose, oropharynx, or trachea should not be performed on a
preventive" schedule.
Kw
96.(D). Aminoglycoside antibiotics should be avoided because they may potentiate the
blocking action of botulinum toxin at the neuromuscular junction.
97.(E). Tetanus is most often generalized but may also be localized. The incubation
period typically is 2-14 days but may be as long as months after the injury. Because
tetanus toxin does not affect sensory nerves or cortical function, the patient
unfortunately remains conscious, in extreme pain, and in fearful anticipation of the
next tetanic seizure. The tetanic paralysis usually becomes more severe in the lst wk
after onset, stabilizes in the 2nd wk, and ameliorates gradually over the ensuing 1-4 wk.
98.(A). Major adverse events include hepatotoxicity in 1% of children and
approximately 3% of adults (increasing with age) and dose-related peripheral
neuropathy. Minor adverse events include rash, worsening of acne, epigastric pain with
occasional nausea and vomiting, decreased vitamin D levels, and dizziness.
99.(B). Aluminum salts decrease absorption of isoniazid, rifampin increase
hepatotoxicity of isoniazid, isoniazid increase level of warfarin and prednisolone
increased isoniazid metabolism.
100.(B). Concentrations of fluconazole are 10-20 fold higher in the urine than blood,
making it an ideal agent for treating fungal urinary tract infections.
101.(E). The cumulative incidence is <O.3% among infants >2,500 g birthweight
admitted to the NlCU. The cumulative incidence increases to 8% for infants <7SO g
birthweight.
101(8). Twice weekly fluconazole at 3 and 6 mg/kg/dose decreases rates of both
colonization with Candidaspecies and invasive fungal infections.
103.(B). While the others inhibits viral DNA polymerase.
104.(B). The use of suppressive therapy with oral acyclovir for 6 mo has been
demonstrated to improve neurodevelopmental outcome.
105.(D). The main route of elimination is renal, and dosage adjustments are necessary
for renal insufficiency. Toxicity is observed typically only in exceptional circumstances:
for example, if administered by rapid infusion to a dehydrated patient or a patient with
underlying renal insufficiency, acyclovir can crystallize in renal tubules and produce a
reversible obstructive uropathy.
27B
106.(D).Ribavirin and its metabolites concentrate in red blood cells and can persistfor
several weeks and, in rare instances, may be associated with anemia.
107.(C).
108.(D). The rash fades over about 7 days in the same progression as it evolved, often
leaving a fine desquamation of skin in its wake.
109.(C). Acute otitis media is the most common complication of measles and
pneumonia is the most common cause of death in measles.
110.(E). Males are affected twice as often as females, and there appear to be more
cases reported from rural than urban populations. Clinical manifestations of SSPEbegin
insidiously 7-13 yr after primary measles infection. The hallmark of the 2nd stage is
massive myoclonus, which coincides with extension of the inflammatory process site to
deeper structures in the brain, including the basal ganglia. The 4th stage is
characterized by loss of critical centers that support breathing, heart rate, and blood
pressure. Death soon ensues.
111.(C). Nerve deafness is the single most common finding among infants with
congenital rubella syndrome.
112.(E). The incubation period for mumps ranges from 12-25 days but is usually 16-18
days. Parotitis may be unilateral initially but becomes bilateral in approximately 70% of
cases. The opening of the Stensen duct may be red and edematous. The parotid
swelling peaks in approximately 3 days and then gradually subsides over 7 days.
113.(A). The most common complications of mumps are meningitis, with or without
encephalitis, and gonadal involvement. Uncommon complications include
conjunctivitis, optic neuritis, pneumonia, nephritis, pancreatitis, and
thrombocytopenia.
114.(E). No fetal malformations have been associated with intrauterine mumps
infection.
115.(E). Symptomatic CNS involvement occurs in 10-30% of infected individuals, but
CSF pleocytosis has been found in 40-60% of patients with mumps parotitis. The
meningoencephalitis may occur before, along with, or following the parotitis. it
most
commonly manifests 5 days after the parotitis. In typical cases, symptoms resolve
in 7-
10 days. The CSF protein content is normal or mildly elevated. Less-common CNS
complications of mumps include transverse myelitis, aqueductal stenosis, and facial
palsy.
116.(A). Hematogenous dissemination of virus to the central nervous system appears
to only occur in neonates.
117.(C). Herpes whitlow is a term generally applied to HSV infection of fingers or toes,
although strictly speaking it refers to HSV infection of the paronychia.
118.(D). HSV is the most common cause of recurrent aseptic meningitis (Mollaret
meningitis).
119.(C). Infants with encephalitis typically present at 8-17 days of life with clinical
findings suggestive of bacterial meningitis, including irritability, lethargy, poor feeding,
279
poor tone, and seizures. Fever is relatively uncommon, and skin vesicles occur in only
approximately 60% of cases.
120.(A). The absolute neutrophil count should be measured at weeks 2 and 4 after
initiation treatment and then monthly.
121. (c). Herpes zoster is caused by the reactivation of latent VZV. it is not common in
childhood and shows no seasonal variation in incidence.
122.(C).The illness usually begins 14-16 days after exposure, although the incubation
period can range from 10-21 days. Subclinical varicella is rare. Temperature elevation is
usually 37.8-38.9(2 but may be as high as 41.1C. Varicella lesions often appear first on
the scalp, face, or trunk.
123.(A). The simultaneous presence of lesions in various stages of evolution is
characteristic of varicella.
124.(E). Clinical recovery is typically rapid, occurring within 24-72 hr, and is usually
complete.
125.(E).
126.(E). EBV is shed in oral secretions consistently for more than 6 mo after acute
infection and then intermittently for life.
127.(E). EBVinfects more than 95% of the worlds population.
128.(8). The classic physical examination findings are generalized lymphadenopathy
(90% of cases), Splenomegaly (50% of cases), and hepatomegaly (10% of cases).
Epitrochlear lymphadenopathy is particularly suggestive of infectious mononucleosis.
Symptomatic hepatitis or jaundice is uncommon, but elevated liver enzymes are very
common. Splenomegaly to 23 cm below the costal margin is typical (15-65% of cases)
and is seen in most cases by ultrasonography; massive enlargement is uncommon.
Palatal petechiae at the junction of the hard and soft palate are frequently seen.
129.(B). Hearing loss is the most common long-term sequela associated with
congenital CMV infection, the failure of an infant to pass a newborn hearing screening
exam should raise the possibility of congenital CMV infection. Hearing loss in the older
infant and young child should also alert the clinician to the possibility of congenital
CMV infection, as approximately 50% of infants with hearing loss associated with
congenital CMV infection will pass an initial hearing screening exam but develop
hearing loss in later infancy and early childhood.
130.(D). Congenital infection with HHV-7 has not been demonstrated.
131.(D).
132.(A).
133.(D). Vaccines are available for HAdV types 4 and 7, but are used only for military
populations.
134.(E). The virus can be identified in 15-20% of children with isolated pharyngitis,
mostly in preschool children and infants.
135.(C).Vomiting and fever typically abate during the 2nd day of illness, but diarrhea
often continues for 5-7 days.
280
136.(Al. Common adverse effects include abdominal pain, diarrhea, and nausea. Rare
side effects include anorexia, flatulence, increased appetite, fever, pruritus, and
dizziness.
137.(A). It is FDA approved for treatment of trichomoniasis and for giardiasis and
amebiasis in children 3 yr of age and older. In the treatment of giardiasis, it has the
advantages of very few side effects and only requiring a single dose. It is excreted via
urine and feces. Tinidazole carries a pregnancy category C classification and can be
detected in breast milk. Breastfeeding should be interrupted during treatment and for
3 days after treatment.
138.(D). Naegleria, Acanthamoeba, Balamuthia, and Sappiniaare small, free living
amebas that cause human amebic meningoencephalitis.
139.(D). The life cycle of G. lamblia (also known as Giardia intestinalis or Giardia
duodenalis) is composed of 2 stages: trophozoites and cysts. Each ingested cyst
produces 2 trophozoites in the duodenum. Giardia trophozoites contain 2 oval nuclei
anteriorly, a large ventral disk, a curved median body posteriorly, and 4 pairs of flagella.
Studies suggest that different Giardia genotypes may cause unique clinical
manifestations, but these findings appear to vary according to the geographic region
tested.
140.(C).
141.(A). Giardiasis should be considered in children who have acute nondysenteric
diarrhea, persistent diarrhea, intermittent diarrhea and constipation, malabsorption,
chronic crampy abdominal pain and bloating, failure to thrive, or weight loss.
142.(D). Asymptomatic excreters generally are not treated except in specific instances
such as outbreak control, prevention of household transmission by toddlers to
pregnant women and patients with hypogammaglobulinemia or cystic fibrosis, and
situations requiring oral antibiotic treatment where Giardia may produce
malabsorption of the antibiotic.Tinidazole not recommended below 3 years old as
albendazole not recommended below age of 6 years. Nitazoxanide1-3 yr: 100 mg bid
for 3 days
143.(C).
14445). Cryptosporidium is recognized as a leading protozoal cause of diarrhea in
children worldwide and is a common cause of outbreaks in childcare centers; it is also a
significant pathogen in immunocompromised patients.
145.(C). The late stage of the illness is often complicated by secondary bacterial
infections, which frequently are a cause of death.
146.(B). Hand-foot-andmouth disease, one of the more distinctive rash syndromes, is
most frequently caused by coxsackievirus A16, sometimes in large outbreaks, and can
also be caused by enterovirus 71; coxsackie A viruses 5, 6, 7, 9, and 10; coxsackie B
Viruses 2 and 5; and some echoviruses.
281
136.(A). Common adverse effects include abdominal pain, diarrhea, and nausea. Rare
side effects include anorexia, flatulence, increased appetite, fever, pruritus, and
dizziness.
137.(A). it is FDA approved for treatment of trichomoniasis and for giardiasis and
amebiasis in children 3 yr of age and older. In the treatment of giardiasis, it has the
advantages of very few side effects and only requiring a single dose. It is excreted via
urine and feces. Tinidazole carries a pregnancy category C classification and can be
detected in breast milk. Breastfeeding should be interrupted during treatment and for
3 days after treatment.
138.(D). Naegleria, Acanthamoeba, Balamuthia, and Sappiniaare small, free living
amebas that cause human amebic meningoencephalitis.
139.(D). The life cycle of G. lamblia (also known as Giardia intestinalis or Giardia
duodenalis) is composed of 2 stages: trophozoites and cysts. Each ingested cyst
produces 2 trophozoites in the duodenum. Giardia trophozoites contain 2 oval nuclei
anteriorly, a large ventral disk, a curved median body posteriorly, and 4 pairs of flagella.
Studies suggest that different Giardia genotypes may cause unique clinical
manifestations, but these findings appear to vary according to the geographic region
tested.
140.(C).
141.(A). Giardiasis should be considered in children who have acute nondysenteric
diarrhea, persistent diarrhea, intermittent diarrhea and constipation, malabsorption,
chronic crampy abdominal pain and bloating, failure to thrive, or weight loss.
142.(D). Asymptomatic excreters generally are not treated except in specific instances
such as outbreak control, prevention of household transmission by toddlers to
pregnant women and patients with hypogammaglobulinemia or cystic fibrosis, and
situations requiring oral antibiotic treatment where Giardia may produce
malabsorption of the antibiotic.Tinidazole not recommended below 3 years old as
albendazole not recommended below age of 6 years. Nitazoxanide1-3 yr: 100 mg bid
for 3 days
143.(C).
144.(E). Cryptosporidium is recognized as a leading protozoa! cause of diarrhea in
children worldwide and is a common cause of outbreaks in childcare centers; it is also a
significant pathogen in immunocompromised patients.
145.(C). The late stage of the illness is often complicated by secondary bacterial
infections,which frequently are a cause of death.
146(8). Handfoot-and-mouth disease, one of the more distinctive rash syndromes, is
rhost frequently caused by coxsackievirus A16, sometimes in large outbreaks, and can
also be caused by enterovirus 71; coxsackie A viruses 5, 6, 7, 9, and 10; coxsackie B
Viruses 2 and S; and some echoviruses.
281
The DigestiveSystem
QUESTIONS
HAI AIMUSAW
arecauses
1.Allthefollowing EXCEPT
dysphagia
oropharyngeal
A. cerebral palsy
B. esophagitis
C. brain tumors
D. cerebrovascular accidents
E. hyperthyroidism
EXCEPT
of emesisin childhood
2. Allthefollowingarecommoncauses
A. gastroenteritis
B. toxic ingestion
C. pertussis syndrome
D. peptic ulcer
E. otitis media
7. Delayed eruption of the primary teeth can be due to the following EXCEPT
A. familial
B. hypopituitarism
C. hyperthyroidism
D. cleidocranialdysplasia
E. trisomy 21
10. Aphthous-like lesions may be associated with the following conditions EXCEPT
A. inflammatory bowel disease
8. Behcet disease
C. gluten-sensitive enteropathy
D. SWEET syndrome
E. herpetic gingivostomatitis
11. Eruption cyst over the erupting tooth is characterized by the following EXCEPT
A. smooth
B. painful
C. blue or blue-black
D. no treatment is indicated
E. resolves after eruption of the tooth
283
must
criteria bemet ofcyclical
definition
fortheconsensus
12Allthefollowing
EXCEPT
syndrome
vomiting
inanyinterval
A. atleast5attacks . 1hrto10
B recurrent ofintense
episodes and
vomitingnausea
lasting and
days
at least1 wk apart
occurring
c. vomiting episodes
during for21hr
24times/hr
occurs
D. returnto baselinehealthbetweenepisodes
E. usuallyattributedto anotherdisorder
17.PierreRobinsyndrome
consists
of the followingEXCEPT
A. mlcrognathia
B. high arched palate
C. cleft palate
D. smalltongue
284
LW
E. foreshortened floor of the mouth
W905? GERD
hiatal hernia
gastric outlet obstruction
285
hasbeen
stenosis
pyloric with EXCEPT
thefollowing
associated
23Hypertrophic
gastroenteritis
A. eosinophilic
B. Apert syndrome
C. Zellwegersyndrome
D. trisomy 21
E. Corneliade Langesyndrome
24.Oralandintravenousatropinesulfate(pyloricmusclerelaxant)hasbeendescribed
treatmentis notavailablefor hypertrophicpyloricstenosiswith a success
whensurgical
rate of
A 20%
B. 40%
C. 60%
D. 80%
E. 90%
25. Meckel diverticulum has been conveniently referred to by the rule of 25, which
explains the classic presentation of this congenital anomaly. One of the following is
FALSE in this rule
A. found in approximately 2% of the general population
B. usually located 2 feet proximal to the ileocecal valve
C. approximately 2 inches in length
D. contain 2 types of ectopic tissue (pancreatic or gastric)
E. found twice as commonly in males
@905? radionuclide
abdominal
abdominal
technetium-99m
ultrasound
CTscan
scans
27. Hirschsprung disease has been seen in association with the following EXCEPT
A. microcephaly
8. mental retardation
C. autism
D. cleft lip
E. hydrocephalus
286
90%
W90?
80%
70%
60%
31. Regarding foreign bodies in the stomach and intestine, all the following are true
EXCEPT
A. coins are the most commonly ingested foreign body
8. perforation is estimated to be <1% of all objects ingested.
C. most objects pass though the intestine in 4-6 days
D. cathartics should be avoided
E. ingestion of batteries usually leads to problems
33, The usual presentingsymptomsof peptic ulcer diseasein infants and younger
children are the following EXCEPT
A. feeding difficulty
287
vomiting
pain
epigastric
FPO? cryingepisodes
hematemesus
common
themost inhumans.
infections
bacterial
34. are
H. Pylori among
A"
thefollowmg
'
aretrueEXCEPT.
rod
S-shaped
isaGram-negative,
A Hpylori can with . pain
manifest . orvomiting
abdominal .
B.H. infection
pylori .
Health
bytheWorld
isclassified
C' H'pylori asagroup
Organization IcafCInogen
isidentified
D'If. pylori notherapy
withnosymptoms,
ina child Should
be
offered . _ .
E H pylori can be associated,though rarely, With chronic autmmmune
thrombocytopenia
35. One of the following features is MORE common in ulcerative colitis than in Crohn
disease
A. abdominal pain
8. growth failure
C. rectal bleeding
D. mouth ulceration
E. strictures
36. A neonate is presented with watery and voluminous stool which is mistaken for
urine.
0f the following,the MOSTlikelycauseis
congenitalglucose-galactosemalabsorption
microvillus inclusion disease
37.TheMOSTcommon
extraintestinal of celiacdiseaseis
manifestation
A. osteoporosis
short stature
arthritis
anemia
iron-deficiency
aphthousstomatitis
288
C. irritability
D. cerebral calcifications
E. cerebellar ataxia
39. The following conditions can be associated with celiac disease EXCEPT
A. Williams syndrome
8. Down syndrome
C. Turner syndrome
D. Klinefilter syndrome
E. Sjogren syndrome
43.Vitamin312andbile saltsareonlyabsorbedin
A. distal ileum
B. duodenum
C. proximaljejunum
D. colon
E. proximal ileum
289
ofthediarrhea
44.Management malnourished
inchronically children
isbased
on
A. intravenoustherapy
osmolarity
standard oralrehydrationsolutions
OS reducedosmolarityoral rehydrationsolutions
D. slow resumptionof feeds
E nothingby mouth for 24 hour
45. The MOST common congenital disorder associated with exocrine pancreatic
insufficiency is
A. Shwachman Diamond syndrome
B. Johanson-Blizzard syndrome
C. Pearson bone marrow syndrome
D. isolated pancreatic enzyme deficiency
E. cystic fibrosis
46. In the blue diaper syndrome, symptoms can include all the following EXCEPT
A. vomiting
B. diarrhea
C. failure to thrive
D. nephrocalcinosis
E. ocular abnormalities
WP???relapsing neuropathy
orange-brown spots on the colon and ileum
increased plasma cholesterol
48. Newborns with congenital chloride diarrhea present with severe life-threatening
secretory diarrhea during the lst few wk of life.
0f the following, the MOSTcommon
laboratory finding is
metabolic alkalosis
hyperchloremia
F909?hyperkalemia
hypernatremia
hypercalcemia
290
D. 2mg/kg/day
E. 2.5 mg/kg/day
wpow» Rotavirus
Giardia
Clostridium difficile
52. Antibiotics are not indicated and may be detrimental in the following foodborne
bacterial illness
A. Enterotoxigenic E. coli
B. Enterohemorrhagic Escherichia coli 01572H7
C. Campylobacterjejuni
D Brucella abortus
E. Bacillusanthracis
54.Appendicitis-likesymptoms(diarrhea,vomiting,fever,andabdominalpain)maybe
seenin the followingfoodbornebacterialillness
A. Vibrio vulnificus
B. Yersinia enterocolitica
C. Shigella spp.
D. Staphylococcus
aureus enterotoxin)
(preformed
E. Listeria monocytogenes
291
age< 9 mo
postmaturity
W909?
if3-36moofage
fever>38°C
diarrhea
secretary
levelofconscnousness
depressed
WorldHealth
56.Thelow-osmolality (WHO)
Organization oralrehydration
solution
has
(ORS) perliterEXCEPT
thefollowing
A. 75 mEqof sodium
8. 50 mEqof chloride
C. 20 mEqof potassium
D. 75 mmol of glucose
E. total osmolarityof 245 mOsm
58. Continuedenteral feeding in diarrhea aids in recovery from the episode, but the
following should be avoided
A. rice
B. potatoes
C. lean meats
D. fruits
E. juices
POP
intestinal inflammation
pancreatic function
reduced intestinal surface
64. The following are alarm symptoms of functional abdominal pain EXCEPT
A. persistent left lower quadrant pain
B. unexplained fever
C. genitourinary tract symptoms
D. dysphagia
E. nocturnal diarrhea
65. A variety of laboratory tests have been used in the evaluation of children with
suspected appendicitis.
Of the following, the MOSTsensitive and specific one is
A. leukocyte count
8. urinalysis
C. electrolytes
D. C-reactive protein
E. serum amyloid A protein
293
maybe helpfulin selected
radiographs
abdominal
66.Plain cases
of abdominal
Plainabdominal
appendicitis. can
x-rays demonstrate
thefollowing
pain/suspected EXCEPT
inacute
findings appendicitis .
ileus
A sentinelloopsof bowelandlocalized
B. scoliosisfrom psoasmusclespasm
C. a colonicair-fluid levelabovethe right iliacfossa
D. a RLQ soft-tissue mass
E. a calcifiedappendicolith(50%of cases)
68. The clinical presentation of one of the following closely mimics appendicitis
A. Meckel diverticulitis
B. mesenteric adenitis
C. sickle cell disease
D. pancreatitis
E. ovarian torsion
wpow
esophageal atresia
spina bifida
tethered cord
72. inguinal hernias are one of the most common conditionsseen in pediatric practice
and the most common surgicalprocedureperformed In pediatricsurgicalpractice.
Regardingthe Incidenceof congenitalindirectinguinalhernia,all the followingare true
EXCEPT
A. approaches30% in verylow birth weight infants
8. more common in boys
C. approximately 60% of inguinal hernias occur on the right side
D. bilateral hernias is higher in boys
E. an increased incidence in twins
ofpancreatic
fordiagnosis
75.Thefirstchoice Is
pseudocysts
ultrasonography
A. transabdominal
B. CT scanning
C. magnetic cholangiopancreatography
resonance
D. endoscopic cholangiopancreatography
retrograde
E. ultrasound
endoscopic
76.Stippied
caicificatlons andgreater
ofthepatellas arefound
trochanter inthe
followingintrahepatlc cholestasis
A. idiopathic
neonatalhepatitis
B. Aagenaes syndrome
C. Zellweger SYN-Ome
(cerebrohepatorenal)
0. neonatal iron storage disease
295
E. Byler disease
rulesoutbiliaryatresia
77.Oneofthefollowing
A. historyof prematurity
8. normal sizeof liver
C. normalconsistencyof liver
D. consistentlypigmentedstools
E. no familial incidence
78. The successrate for establishinggood bile flow after the Kasaioperation is much
higher (90%)if performed before
A. 4 wk of life
B. 6 wk of life
C. 8 wk of life
D. 10 wk of life
E. 12 wk of life
79. For patients with advanced liver disease, hepatic transplantation has a success rate
>85%. If the operation is technically feasible, it will prolong life and might correct the
metabolic error in diseases such as
A. tyrosinemia
B. galactosemia
C. hereditary fructose intolerance
D. Zellwegersyndrome
E. Crigler-Najjar
80. Persistence of unconjugated hyperbilirubinemia at levels >20 mg/dL after the lst
wk of life in the absence of hemolysis should suggest
A galactosemia
B. tyrosinemia
C. al-Antitrypsin deficiency
D. Crigler-Najjar type I
E. Maple serum urine disease
296
82. Kayser-Fleischer
(K-F) ring is a brown discolorationat the outer marginof the
cornea;the following are true EXCEPT
A. might not be presentin youngerchildren
3. requires a slit-lamp examination
C. is permenant even after trearment
D. is present in 95% of patientswith
neurologicsymptoms
E. is deposition of copper in Descemets membrane
83. A major attempt in Wilson disease should be made to restrict dietary copper intake
to <1 mg/day.
Of the following, the food that should be avoided is
A. liver
8. meat
C. egg
0. chicken
E fish
84. Neonatal iron storage disease (NISD), also known as neonatal hemochromatosis is a
rapidly fatal, progressive illness characterized by the following EXCEPT
hypoprothrombinemia
hypoalbuminemia
W905?hyperferritinemia
hyperbilirubinemia
hyperglycemia
in
86. HBVispresentin highconcentrations
A. serous exudates
B. saliva
C. vaginal fluid
D. semen
E. urine
87.Risk inchildren
for HBVinfection
factors include
andadolescents bythe
acquisition
followingEXCEPT
A. intravenous drug
297
contaminatedneedles
sexual contact
app?
intimatecontactwith carriers
sharing toys
88.Regarding
epidemiology aretrueEXCEPT
of HBV,thefollowing
intrauterine infection occursin 2.5%
antigenemiaappear1-3 mo after birth
89. Routine screeningfor HBV infection requires assayof multiple serologic markers,all
the following are true EXCEPT
A. HBsAgis the first serologic marker of infection to appear
8. Anti-HBc is a valuable serologic marker of acute HBV infection
C. anti-H85 and anti- HBc are detected in persons immunized with hepatitis B
vaccine
D. HBeAg is a marker of infectivity
E. HBsAg levels fall before symptoms wane
91. The MOST common hepatobiliary disease associated with inflammatory bowel
disease (IBD) is
A. hepatic abscess
8. portal vein thrombosis
C. sclerosing cholangitis
D. autoimmune hepatitis
E. biliary carcinoma
92. Totalparenteralnutrition(TPN)cancausea
variety of liver diseases.
Of the following,the MOSTsevere
complicationis
A hepatic steatosis
B. gallbladder damage
C. bile duct damage
D. cholestasis
E. sclerosing cholangitis
["9093
presence of autoantibodies
characteristic histologic findings
POP
E.
cirrhosis
cholestasis
Wilson disease
95. Biliary sludge or cholelithiasis can be detected in >40% of children who are treated
with prolonged course of
cefotaxime
W909"?
meropenem
vancomycin
erythromycin
ceftriaxone
299
98.Ascitesisthepathologic of fluidwithinthe peritonealcavity.
accumulation
Ofthefollowing,the MOSTcommoncausein childrenis
A. neoplasia
B. hepatic
C. cardiac
D. trauma
E. infection
99. Primary peritonitis usually refers to bacterial infection of the peritoneal cavity
without a demonstrable intraabdominal source.
Of the following, the MOST common isolated bacteria are
A. groupAstreptococci
B. Staphylococci
C. Escherichia coli
D. Mycobacterium tuberculosis
E. Pneumococci
100. Acute secondary peritonitis most often results from entry of enteric bacteria into
the peritoneal cavity through a necrotic defect in the wall of the intestines or other
viscus.
It MOST commonly follows
A. perforation of the appendix
B. rupture of a Meckel diverticulum
C. intussusception
D. peptic ulceration
E. necrotizing enterocolitis
The Digestive System
ANSWERS
301
15.(D).Delayederuption of the 20 primary teeth can be familial or indicate systemicor
nutritional disturbances such as hypopituitarism, hypothyroidism, cleidocrania|
dysplasia,trisomy 21, and multiple syndromes.
16.(A). Mottled enamel (permanent teeth) is seen in kidney failure and cystic fibrosis.
17(0). The tongue is usually of normal size, but the floor of the mouth is
foreshortened. The air passages can become obstructed, particularly on inspiration,
usually requiring treatment to prevent suffocation.
18.(E). It is uncommon before school age.
19.(C). Contrast (usually barium) radiographic study of the esophagus and upper
gastrointestinal tract is performed in children with vomiting and dysphagia to evaluate
for achalasia, esophageal strictures and stenosis, hiatal hernia, and gastric outlet or
intestinal obstruction. It has poor sensitivity and specificity in the diagnosis of GERD.
20.(B). Infantile refluxmanifests more often with regurgitation(especia|ly
postprandially), signs of esophagitis (irritability, arching, choking, gagging, feeding
aversion), and resulting failure to thrive; symptoms resolve Spontaneously in the
majority of infants by 12-24 mo.
21.(E). Seated position worsens infant reflux and should be avoided in infants with
GERD.
22.(A). More common in boys.
23.(D). Trisomy 18.
24(0).
25.(E). Meckel diverticulum is found twice as commonly in females.
26.(C). The most sensitive study is a Meckel radionuclide scan, which is performed after
intravenous infusion of technetium-99m pertechnetate. The mucus-secreting cells of
the ectopic gastric mucosa take up pertechnetate, permitting visualizationof the
Meckel diverticulum. The uptake can be enhancedwith various agents, including
cimetidine, ranitidine, glucagon, and pentagastrin. The sensitivity of the enhanced scan
is approximately 85%, with a specificity of approximately 95%. A false-negative scan
may be seen in anemic patients; although false-positive results are uncommon, they
have been reported with intussusception, appendicitis, duplication cysts, arteriovenous
malformations, and tumors.
27.(D). Cleft palate
28.(A). in 99% of healthy full-term infants, meconium is passed within 48 hr of birth.
29.(C). in children, it is most often associated with abdominal surgery or infection
(gastroenteritis, pneumonia, peritonitis). lleus also accompanies metabolic
abnormalities (e.g., uremia, hypokalemia, hypercalcemia, hypermagnesemia, and
acidosis)or administration of certain drugs, such as opiates, vincristine, and antimotility
agents such as loperamide when used during gastroenteritis.
30(8). The classictriad of pain, a palpable sausage-shapedabdominal mass, and bloody
or current jelly stool is seen in <30% of patients with intussusception.
31.(E). Ingestion of batteries rarely leads to problems, but symptoms can arise from
leakage of alkali or heavy metal (mercury) from battery degradation in the
302
gastrointestinal tract. Batteries can also generate electrical current and thereby cause
low-voltage electrical burns to the intestine.
3245). Sunflower seed bezoars are reported to cause rectal pain and constipation as a
result of the seed shells being associated with fecal impaction. Endoscopic removal is
indicated, as these bezoars are refractory to enema or lavage management.
33.(C). School-age children and adolescents more commonly present with epigastric
pain and nausea, presentations generally seen in adults.
34(0). if H. pylori is identified, even in a child with no symptoms, eradication therapy
should be offered
354C), Rectal bleeding, diarrhea, mucus, pus, rectal involvement, toxic megacolon,
sclerosing cholangitis, and crypt abscesses are more common in ulcerative colitis.
36.(8). in secretory diarrhea caused by disorders such as congenital chloride diarrhea
and microvillus inclusion disease, the stool is watery and voluminous and can be
mistaken for urine.
31(0). The most common extraintestinal manifestation of celiac disease is iron-
deficiency anemia, unresponsive to iron therapy. Osteoporosis may be present; in
contrast to the situation in adults, it can be reversed by a gluten-free diet, with
restoration of normal peak bone densitometric values. Other extraintestinal
manifestations include short stature, arthritis and arthralgia, epilepsy with bilateral
occipital calcifications,peripheral neuropathies, cardiomyopathy, isolated
hypertransaminasemia, dental enamel hypoplasia, aphthous stomatitis, and alopecia.
38.(A). Peripheral neuropathy.
39.(D). Some diseases, many with an autoimmune pathogenesis, are found with a
higher-than-normal incidence in celiac disease patients. Among these are type 1
diabetes, autoimmune thyroid disease, Addison disease, 5], gren syndrome,
autoimmune cholangitis, autoimmune hepatitis, primary biliary cirrhosis. Such
associations have been interpreted as consequence of the sharing of identical HLA
a
haplotypes. The relation between celiac disease and other autoimmune diseases is
poorly defined; once those diseases are established, they are not influenced by a
gluten-free diet. Other associated conditions include selective IgA deficiency and Down,
Turner, and Williams syndromes.
40(5). intellectual development tends to be slow.
41.(C). Malabsorption is mainly seen after infection with Campylobacter, Shigella,
Salmonella, Giardia, cryptosporidium, coccidioidosis, and rotavirus. These infectious
causes of malabsorption are more common in immunocompromisedchildren.
42.(A). The pathogenesis of the diarrhea is not always clear and may be related to
secondary lactase deficiency, food protein allergy, antibiotic-associated colitis
(including pseudomembranous colitis caused by Clostridiumdifficlle toxin), or a
combination of these.
431A). Vitamin 812 and bile salts are only absorbed in the distal ileum.
303
44.(C).Managementof the diarrhea In chronically malnourished children is basedon 3
principles: oral rehydration to correct dehydration, rapid resumption of feeds with
avoidanceof periods of nothing by mouth, and treating the etiology of the diarrhea.
45(5). Cystic fibrosis is the most common congenital disorder associated with exocrine
pancreatic insufficiency.
4648). Symptoms can include digestive disturbances such as vomiting, constipation,
poor appetite, failure to thrive, hypercaicemia, nephrocaicinosis, fever, irritability, and
ocular abnormalities.
47.(E). Features of Tangier disease include orange tonsils, hepatospienomegaiy,
relapsing neuropathy, orange-brown spots on the colon and ileum, diarrhea in
association with decreased plasma cholesterol levels (apollpoprotein A-i and A-ll), and
normal or elevated triglyceride levels. Specific therapy for Tangier disease has not yet
been established.
48.(A). Laboratory findings are metabolic alkaiosls, hypochioremia, hypokalemia, and
hyponatremla (with high plasma renln and aldosterone activities).
4948). Acrodermatitls enteropathica requires long term treatment with elemental zinc
1 mg/kg/day.
SO.iA). Direct person-to-person contact outbreaks of gastroenteritis are usually caused
by norovlrus and Shlgelia species. Unknown agents are seen in 30-40%; other
pathogens include Salmonella, rotavirus, Giardla, Cryptosporidium, Ciostridium difficile,
and C. Jejuni.
51.(D). Treatment of Campyiobacter JeJuni is supportive care.For severe cases,
antibiotics, such as azlthromycin and quinolones, may be indicated early in the
diarrheal diseaseGuillain-Barré syndrome can be a sequel.
5248). Treatment of Enterohemorrhaglc Escherichia coli(EHEC) E. coil 0157:H7 include
supportive care, monitor renalfunctlon, hemoglobin, and platelets closely. E. coil
01571H7infection is also associated with hemolytic uremic syndromeiHUS), which can
cause lifelong complications. Studies indicate that antibiotics might promote the
development of HUS. Antidiarrheal agents like lmodium may also increase the risk of
developing HUS.
53.(A). Treatment of Vibrio cholerae is supportive care with aggressive oral and
intravenous rehydration.Doxycycline is recommended as first-line treatment for adults,
whereas azlthromycin is recommended as first-line treatment for children and
pregnant women. Ciprofloxacin and doxycycllne recommended as second-line drugs for
children.
5443). Signs and symptoms of Yerslnla enterocolltlca and Yerslnia pseudotubercuiosis
are, appendicitis-like symptoms (diarrhea and vomiting, fever, abdominal pain) occur
primarily in older children and young adults; might have a scarlatiniform rash or
erythema nodosum with Y.Pseudotuberculosis.
554$). Risks associated with severe dehydration that might necessitate intravenous
resuscitation include: age <6 mo; prematurity; chronic illness; fever >38'C (100.4F) if
younger than 3 mo or >39°C (102.2F) if 3-36 mo of age; bloody diarrhea; persistent
emesis; poor urine output; sunken eyes;and a depressed level of consciousness.
56.(B). The |ow~osmolality World Health Organization (WHO) oral rehydration solution
(ORS) containing 75 mEq of sodium, 64 mEq of chloride, 20 mEq of potassium, and 75
mmol of glucose per liter, with total osmolarity of 245 mOsm/L, isnow the global
standard of care and more effective than home fluids, including decarbonated soda
beverages, fruit juices, and tea. These are not suitable for rehydration or maintenance
therapy because they have inappropriately high osmolalities and low sodium
concentrations.
57.(D). Characterized by recurrent episodes of blood in the urine, this condition results
from deposits of the protein lgA in the glomeruli. IgA nephropathy can progress for
years with no noticeable symptoms. Men seem more likely to develop this disorder
than women.
58.(E). Fatty foods or foods high in simple sugars (juices, carbonated sodas) should be
avoided.
59.(A). Administration of zinc in community settings leads to increased use of ORS and
reduction in the inappropriate use of antimicrobials.
60.(D). Because persistent vomiting can limit oral rehydration therapy, a single
sublingual dose of an oral dissolvable tablet of ondansetron (4 mg 4-11 yr and 8 mg for
children older than 11 yr [generally 0.2 mg/kg]) may be given.
61.(C). Nitazoxanide, an antiinfective agent, is effective in the treatment of a wide
variety of pathogens, including C. parvum, G. lamblia, E. histolytica, Blastocystis
hominis, C. difficile, and rotavirus.
62(8). The drug of choice for shigella is Ciproiioxacin, ampicillin, ceftriaxone,
azithromycin, or TM P-SMX.
63.(C). Abnormalities in the digestiveabsorptive function tests suggest small bowel
involvement, whereas intestinal inflammation, as demonstrated by increased fecal
calprotectinor lactoferrin, supportscolitis.
64.(A). Pain that wakes up the child from sleep, persistent right upper or right lower
quadrant pain.
65.(E). Serum amyloid A protein is consistently elevated in patients with acute
appendicitiswith a sensitivityand specificityof 86% and 83%, respectively.
66.(E). A calcified appendicolith (540% of cases),
67(8). Decreased local tenderness to compression.
88.(A). Meckel diverticulitis is an infrequent condition, butthe clinical presentation
closelymimics appendicitis and the diagnosis is usually made at surgery.
69.). There are many anomalies associated with anorectal malformations. The most
common are anomalies of the idneys and urinary tract in conjunction with
abnormalitiesof the sacrum. This co lex is often referred to as the caudalregression
Syndrome.
70.(C).100% in Familialadenomatouspolyposis(FAP)and Gardnersyndrome.
305
71.(E).Polypsmay be found anywhere in the gastrointestinal (GI) tract, most commonly
in the colon or rectum; they are often solitary but may be multiple.
72.(D).The incidence of bilateral hernias is higher in girls and appears to be 20-40%.
73.(D). The features of the Johanson-Blizzard syndrome include exocrine pancreatic
deficiency, aplasia or hypoplasia of the alae nasi, congenital deafness, hypothyroidism,
developmental delay, short stature, ectodermal scalp defects, and absence of
permanent teeth, urogenital malformations, and imperforate anus.
74.(B). Serum lipase is now considered the test of choice for acute pancreatitis as it is
more specific than amylase for acute inflammatory pancreatic disease and should be
determinedwhen pancreatitis is suspected.
75.(A). Because of its ease, availability, and reliability, ultrasonography is the first
choice.
76.(C). Affected infants have severe, generalized hypotonia and markedly impaired
neurologic function with psychomotor retardation. Patients have an abnormal head
shape and unusual facies, hepatomegaly, renal cortical cysts, stippled calcifications of
the patellas and greater trochanter, and ocular abnormalities.
77.(D). Consistently pigmented stools rule against biliary atresia. The finding of bile-
stained fluid on duodenal intubation also excludes biliary atresia.
78.(C). The success rate for establishing good bile fiowafter the Kasai operation is much
higher (90%) if performed before 8 wk of life. Therefore, early referral and prompt
evaluation of infants with suspected biliary atresia is important.
79.(A). it will prolong life and might correct the metabolic error in diseases such as (11-
antitrypsin deficiency, tyrosinemia, and Wilson disease.
80.(D). The diagnosis of CN type i is based on the early age of onset and the extreme
level of bilirubin elevation in the absence of hemolysis. In the bile, bilirubin
concentration is <10 mg/dL compared with normal concentrations of 50-100 mg/dL;
there is no bilirubin glucuronide. Definitive diagnosis is established by measuring
hepatic glucuronyl transferaseactivity in a liver specimen obtained by a closed biopsy.
81.(A). Liver histology demonstrates normal architecture, but hepatocytes contain
black pigment similar to melanin. Liver function is normal and prognosis is excellent.
The most commonly reported symptoms are abdominal pain and fatigue, jaundice,
dark urine, and slight enlargement of the liver. Jaundice fluctuates in intensity and is
aggravated by intercurrent disease.
82.(C). After adequate treatment, Kayser-Fieischer rings resolve.
83.(A). A major attempt should be made to restrict dietary copper intake to <1 mg/day.
Foods such as liver, shelmsh, nuts, and chocolate should be avoided.
SHE). NISD is a rapidly fatal, progressive illness characterized by hepatomegaly,
hypoglycemia, hypoprothrombinemia, hypoalbuminemia, hyperferritinemia, and
hyperbilirubinemia. The coagulopathy is refractory to therapy with vitamin K. Liver
pathology demonstrates severe liver injury with acute and chronic inflammation,
fibrosis, and cirrhosis.
8$.(E). Rare in HAV, common in HBV, HCV, and HDV, never in HEV.
864A). HBV is present in high concentrationsin blood, serum, and serousexudates and
in moderate concentrations in saliva, vaginal fluid, and semen.
81(6). HBV is not thought to be transmitted via indirect exposure, such as sharing toys.
8840). Breastfeeding of nonimmunized infants by infected mothers does not confer a
greater risk of hepatitis than does formula feeding.
89.(C). Only anti-H85 is present in persons immunized with hepatitis B vaccine, whereas
both anti-HBs and anti HBc are detected in persons with resolved infection.
90.iE). HBV is not spread by breastfeeding, kissing,hugging, orsharing water or utensils.
91.(C). Sderosing cholangitisis the most common hepatobiliarydiseaseassociatedwith
IBD, ocwrn'ng in 2-896 of adult patients with ulcerative colitis and less often in Crohn
disease.
92.(D). Cholestasisis the most severe complication and can lead to progressive fibrosis
and cirrhosis. it is the major factor limiting effective long-term use of TPN in children
and adults. Risk factors for TPN-associated cholestasisinclude prolonged duration of
TPN, prematurity, low birthweight, sepsis, necrotizing enterocolitis, and short bowel
syndrome.
93.13). Primary elevation in transaminases and not alkaline phosphatase.
94.(B). Hypoplasia or absence of the gallbladder an be associated with extrahepatic
biliary atresia or cystic fibrosis.
95.(E). Prolonged use of high-dose oeftriaxone, a thirdgeneration cephalosporin, has
been associated with the formation of calcium-ceftriaxone salt precipitates (biliary
pseudolithiasis) in the gallbladder. Biliary sludge or cholelithiasis can be detected in
>40% of children who are treated with ceftriaxone for at least 10 days. in rare cases,
children become jaundiced and develop abdominal pain; precipitates usually resolve
spontaneouslywithin several months after discontinuation of the drug.
961C). Bleeding from esophageal varices is the most common presentation.
Splenomegaly,sometimes with hypersplenism,is the next most common presenting
feature in portal vein obstruction and may be discovered first on routine physical
examination.
9ND). Biliary atresia is the most common indication for liver transplantation in
,children, followed by metabolic and inborn disorders, autoimmune and familial
cholestaticdisorders, and acute hepatic necrosis.
9848). in children, hepatic and renal diseasesare the most common causes.
99.(E). Pneumococci(most common), group A streptococci,enterococci, staphylococci,
and Grammegative enteric bacteria, especially Escherichia coli and Klebsiella
pneumoniae, are most commonly found. Mycobacterium tuberculosis, Neisseria
Meningitidis,and Mycobacterium bovis are rare causes.
wow. it most commonly follows perforation of the appendix. Other causes include
incarcerated hernias, rupture of a Meckel diverticulum, midgut volvulus,
hemolyticuremic syndrome,peptic ulceration,inflammatorybowel
intuSsusception,
disease, necrotizing cholecystitis, necrotizing enterocolitis, typhlitis, and traumatic
bedoration.
- System
Respiratory
QUESTIONS
ALMUSAWI
ZUHAIR
against
byexpiration
isproduced closed
a partially glottisandisanattempt
1.Grunting
tomaintain
positive pressure
airway foraslong
expiration
during aspossible.
it isMOSTcommonly in
prominent
A. epiglottitis
B. hyalinemembranedisease
C. asthma
D. croup
E. choanalatresia
PROPS?
4.Rigid
Pulmonaryembolisms
vascular rings
bronchiectasis
ispreferentially
indicated
for
A. bronchoscopy
persistentpneumonia
8- extracting
foreignbodies
C. atelectasis
9- persistentwheeze
E. interstitial
disease
C. pertussis
D. Staph. pneumonia
E. sepsis
coldtreatmentEXCEPT
10.Thefollowingmaybe effectiveasa common
A. vitamin C
8. first generationantihistamine
C. topical or oral adrenergicagents
D. ipratropium bromide
E. honey
of the [hternai
by septicthrombophlebitis
is characterized
syndrome
11.Lemierre emboli,producing and
hypoxia
jugular veins . .
pulmonary
withseptic FU'monary
causedby
n of pharyngitis
infiltrates, it isacomplicatio
A
streptococcus
groupA Bhemolytic
B. haemolyticum
Arcanobacterium
C. pneumoniae
Mycoplasma
D. necrophorum
Fusobacterium
E. diphtheriae
Corynebacterium
FUDF
stomach
nose
14. A silent chest with infiltrates should arouse suspicion of all the following EXCEPT
alveolar proteinosis
bronchopulmonary dysplasia
310
severe stridor at rest
FPO?
cyanosis
depressed mental status
congenital heart disease
18. The MOST common congenital laryngeal anomaly that produces stridor is
A. vocal cord paralysis
B. laryngomalacia
C. congenital subglottic stenosis
D. congenital laryngeal web
E. congenital subglottic hemangioma
19. Risk factors for persistent wheezing include all the following EXCEPT
A. parental history of asthma
B. paternal smoking
C. persistent rhinitis (apart from acute upper respiratory tract infections)
D. eczema at <1 yr of age
E. frequent episodes of wheezing during infancy
91.005"
right ventricular enlargement
left ventricular enlargement
coarctation of aorta
21.Regarding lobaremphysema
congenital aretrueEXCEPT
thefollowing
(CLE),
A. familialoccurrencehasbeenreported
no cause
3. usually canbeidentified .
ofCLE
become
C. usually intheneonatal
apparent period
0- many arediagnosed
cases ultrasonography
byantenatal
site
E. leftupperlobeisthemostaffected
311
(BO)is a rarechronicobstructive
obliterans
22.Bronchiolitis lungdisease of the
An insultto the lowerrespiratory
andsmallerairways.
bronchioles tract occurs,
resultingin fibrosisof the smallairways.
meanof establishing
the BEST
0f thefollowing, is
its diagnosis
A. chest radiographs
B. pulmonary function tests
C. ventilation-perfusion scans
D. chest CT
E. open lung biopsy
24. The recurrent aspiration of small quantities of gastric, nasal, or oral contents can
lead to several clinical presentations, including recurrent bronchitis or bronchiolitis;
recurrent pneumonia; atelectasis; wheezing; cough; apnea; and/or laryngospasm.
The MOST common underlying problem associated with recurrent pneumonias in
hospitalized children is
A. oropharyngealincoordination
B. esophageal foreign body
C. nasoenterictube
D. poororal hygiene
E bronchopulmonary dysplasia
312
C. group A streptococci
D. H. influenzae(type b, nontypable)
E. adenovirus
F1909"?
tachypnea
fever
cough
313
syndromes
deficiency
D. immune
E. cysticfibrosis
complaint
common
MOST is
withbronchiectasis
inpatients
32The of sputum
purulent
andproductioncopious
A. cough
B. hemoptysis
C. fever
0. anorexia
E. poor weight gain
fibrosis
33.Cystic (CF) EXCEPT
withallthefollowing
canbepresented
A salt depletion
B. pansinusitis
C. rectal polyposis
D. pancreatitis
E. cholelithiasis
37.Regardingcysticfibrosis,all the
following are true EXCEPT
A. delayed sexual
development
B. morethan 95%of malesare
azoospermic
C. sexualfunction is
generallyimpaired
314
D. high incidence of inguinal hernia
5, diminished female fertility rate
38- f°0Wing
conditions
areassociated
withfalse-positive
sweattestresults
:élrthe
EXC
eczema
anorexia nervosa
W909? hypothyroidism
Klinefelter syndrome
malnutrition
39. The finding of the following pathogen on culture of the lower airways (Sputum)
strongly suggests a diagnosis of cystic fibrosis
A. B. cepacia
B. gram-negative rods
C. nontuberculous mycobacterial species
D. Mycoplasma
E. S. aureus
40. All the following inhalation therapies in cystic fibrosis are true EXCEPT
A. B-agonists may decrease P302 acutely
B. human recombinant DNase improves pulmonary function
C. Nacetylcysteine is toxic to ciliated epithelium
D. nebulized hypertonic saline improves mucociliary clearance
E. aerosolized antibiotics are often used when the airways are colonized with S.
aureus
41. The following are clinical manifestations of primary ciliary dyskinesia (PCD) EXCEPT
A. recurrent pneumonia
8. chronic otitis media
C. chronic pansinusitis
D. dextrocardia
E. retinitis pigmentosa
315
hemosrderosusin assocnatlonwuth cow's
lo3ic findi ngs of pulmonary
43.Path0 . milk
includea thefollowmg EXCEPT
hypersensitivity
elevationsof lgA
eosinophilia
peripheral
W995?
of lgG,lgA
deposits
alveolar
alveolardepositsof C3
hightiterstocow'smilkprotein
ofidiopathic
hndings
laboratory
44.Theclassic hemosiderosis
pulmonary (IPH)arethe
followingEXCEPT .
A. microcytichypochromicanemia
B. elevated reticulocyte count
C. normal plasmabilirubin
D. reduced serum iron
E. elevated ironbinding capacity
£71,005?
cyclophosphamide
chloroquine
lung transplantation
46. A commonly encountered risk factor for deep venous thrombosis (DVT) and
pulmonaryembolism (PE)in the pediatric population is
A central venous catheter
B. hematologic malignancies
C. sickle cell diease
D. nephrotic syndrome
E. antiphospholipid
antibody syndrome
47.TheMOSTcommonform of
pulmonarymalignancyin childrenis
A. bronchial carcinoid
8 . adenoid
cystic carcinoma
C. mucoepidermoid
carcinoma
D. metastatic lesions
E.
pulmonary blastoma
316
C. bacterial pneumonia
D. rheumatoid arthritis
E. metastatic intrathoracic
malignancy
TheMOSTcommon
cause
of pneumomediastinum andteenagers
inolderchildren
9.
lS
dental extractions
adenotonsillectomy
FPDWPtuberculosis
mycoplasma pneumonia
acute asthma
inchildren
likelycauseof hemothorax
the LEAST
52.Ofthefollowing, is
A. chest trauma
8. intrathoracic neoplasms
C. costal exostoses
D. blood dyscrasias
E. rupture of an aneurysm
EXCEPT
aretrueinchylothorax
53.Allthefollowing
A. fluid may be clear
level
3- fluidtriglyceride is>110mg/dL
C. pleuralfluid:serum ratiois>1.0
triglyceride
D. pleuralfluidzserum ratiois<1.0
cholesterol
Blymphocytes
E. cellsareprimarily
317
(BPD)
dyspiasia
54 Bronchopulmonary isa syndromecharacterized
bySigns
and
ofchronic that
disease
lung In
originatestheneonatal An
period.accepted
symptoms for
requirement
oxygen
a
includes
definition postnatal
A. 7days
B. 14 days
C. 21 days
D. 28 days
E. 35 days
55.TheMOSTcommon
physical examin bronchopulmonary
findingof the pulmonary
dyspiasia(BPD)is
tachypnea
mouth breathing
57. The following are features of pectus excavatum (funnel chest) EXCEPT
A. occurs in 1:400 births
8. usually associated with a connective tissue disorder
C. 9: 1 male preponderance
D. accounts for >90% of congenital chest wall anomalies
E. positive family history in one third of cases
Acute
W909? high familial occurrence
common association of mild to moderate
association with mitral valve disease
manifestation
clinical
scoliosis
5? respiratory
o
isoften
theMOST
insufficiency prominent
A. Duchennemuscular
dystrophy
318
B. Spinalmuscular atrophy
C. congenitalmyotonic dystrophy
D. myasthenia gravis
E. Guillain-Barre syndrome
60'Asphyxiating
thoracic
dVSt'OIDhY
(Jeune withthefollowing
isassociated
syndrome)
skeletal abnormalities EXCEPT
narrowed thorax
horizontal ribs
319
- Respiratory
System
ANSWERS
ZUHAIR ALMUSAWI
1.(B). Grunting is most beneficial in alveolar diseases that produce widespread lossof
FRC, such as in pulmonary edema, hyaline membrane disease, and pneumonia.
Grunting is also effective in small airway obstruction (bronchiolitis) to maintain a higher
positive pressure in the airway during expiration, decreasing the airway collapse.
2.(A). The PVR is ~50% of the systemic arterial resistance 3 days after birth. in the next
several wk after birth as pulmonary arterial musculature in the tunica media involutes,
there is a further decline in PVR and therefore in pulmonary artery pressure. Two to
3 mo after birth, the PVR and the pulmonary artery pressure are ~15% of the systemic
values, a relationship that exists through childhood and adolescence.
3.(D). MRI is an excellent procedure to delineate hilar and vascular anatomy associated
with vascular rings or slings.
4(8). Rigid bronchoscopy is preferentially indicated for extracting foreign bodies, for
removing tissue masses, and in patients with massive hemoptysis. In other cases, the
flexible scope offers the advantages that it can be passed through endotracheal or
tracheostomy tubes can be introduced into bronchi that come off the airway at acute
angles,and can be safely and effectively inserted with topical anesthesia and conscious
sedation.
5.(D). The following infections can cause sudden death in infants: sepsis, meningitis,
encephalitis, brain abscess,hepatitis, pyelonephritis, bronchiolitis (respiratory syncytial
virus), infant botulism, and pertussis.
6.(A). The reductions in risk appear to be related primarily to decreases in placing
infants prone for sleep and increases in placing them supine. A number of other risk
factors also have significant associations with SIDS; although many are nonmodifiable
and most of the modifiable factors have not changed appreciably, self-reported
maternal smoking prevalence during pregnancy has decreased by 25% in the past
decade.
7.(A). Presentingclinicalsymptomsincludehistory of insertion of foreign bodies(86%),
mucopurulent nasal discharge (24%), foul nasal odor (9%), epistaxis (6%), nasal
obstruction(3%), and mouth breathing (2%).
8.(C). Common causesof nosebleeds from the anterior septum include digital trauma,
foreign bodies, dry air, and inflammation, including upper respiratory tract infections,
sinusitis, and allergic rhinitis. Young infants with significant gastroesophageal reflux
into the noserarely present with epistaxis secondary to mucosal inflammation.
9.(E).Cysticfibrosisis the most commonchildhoodcause of nasal polyposisand should
be suspectedin any child <12 yr old with nasal polyps,even in the absenceof typical
320
respiratory and digestive symptoms; as many as 30% of children with cystic fibrosis
acquire nasal polyps.
10.(A). Vitamin C, guaifenesin, and inhalation of warm, humidified air are no more
effective than placebo for the treatment of cold symptoms.
11.(D). Lemierre syndrome is a serious complication of F. necrophorum pharyngitis and
is characterized by septic thrombophlebitis of the internal jugular veins with septic
pulmonary emboli, producing hypoxia and pulmonary infiltrates.
12.(D). Because cough receptors also reside in the pharynx, paranasal sinuses, stomach,
and external auditory canal, the source of a persistent cough may need to be sought
beyond the lungs.
13.(D). Habit cough (psychogenic cough" or cough tic) must be considered in any
child with a cough that has lasted for weeks or months, that has been refractory to
treatment, and that disappears with sleep or with distraction.
14(8). A "silent chest" with infiltrates should arouse suspicion of alveolar proteinosis,
Pneumocystis jiroveci infection, genetic disorders of surfactant synthesis and secretion
causing interstitial pneumonitis, or tumors.
15.(B). This dramatic, potentially lethal condition is characterized by an acute rapidly
progressive and potentially fulminating course of high fever, sore throat, dyspnea, and
rapidly progressing respiratory obstruction. The barking cough typical of croup is rare.
16.(E). Children with croup should be hospitalized for any of the following: progressive
stridor, severe stridor at rest, respiratory distress, hypoxia, cyanosis, depressed mental
status, poor oral intake, or the need for reliable observation.
17.(D). Bacterial tracheitis often follows a viral respiratory infection (especially
laryngotracheitis), so it may be considered a bacterial complication of a viral disease,
rather than a primary bacterial illness.
18(8). Laryngomalacia is the most common congenital laryngeal anomaly and the most
common cause of stridor in infants and children. Sixty percent of congenital laryngeal
anomalies in children with stridor are due to laryngomalacia.
19(8). Multiple studies have tried to predict which early wheezers will go on to have
asthma in later life. Risk factors for persistent wheezing include parental history of
asthma and allergies, maternal smoking, persistent rhinitis (apart from acute upper
respiratory tract infections), eczema at <1 yr of age, and frequent episodes of wheezing
during infancy.
20.(B). Cardiovascular causes of wheezing include dilated chambers of the heart
including massive cardiomegaly, left atrial enlargement, and dilated pulmonary
arteries.
21(3). in 50% of cases, a cause of CLE can be identified. Congenital deficiency of the
bronchial cartilage, external compression by aberrant vessels, bronchial stenosis,
redundant bronchial mucosal flaps, and kinking of the bronchus caused by herniation
into the mediastinum have been described as leading to bronchial obstruction and
subsequent CLE and commonly affects the left upper lobe.
321
22.(E).Open lung biopsy or transbronchial biopsy remains the best means of
establishingthe diagnosisof BC.
23(0). Air bronchogramsis usually present in non cardiogenic edema while the other
findingsare usuallyfound in cardiogenic pulmonary edema.
24.(A). Oropharyngeal incoordination is reportedly the most common underlying
problem associatedwith recurrent pneumonias in hospitalized children. In one series of
238 children hospitalized with recurrent pneumonia, 48% were found to have
dysphagiaasthe underlying problem.
25.(E).The most common etiology of PIE syndromes includes parasite infections and
drug reactions.
26(8). Streptococcus pneumoniae (pneumococcus) is the most common bacterial
pathogen in children 3wk to 4yr of age, whereas Mycoplasma pneumoniae and
Chlamydophila pneumoniae are the most frequent pathogens in children Syr and
older.
27.(E).The most frequent pathogens of pneumonia in children 4 mo-4 yr (in order of
freguency) are Respiratory syncytial virus, other respiratory viruses (parainfluenza
viruses, influenza viruses, and adenovirus), S. pneumoniae, H. influenzae (type b,
nontypable), Mycoplasma pneumoniae, and group A streptococcus.
28.(A). Recurrent pneumonia is defined as 2 or more episodes in a single year or 3 or
more episodes ever, with radiographic clearing between occurrences.
29.(C).Tachypnea is the most consistent clinical manifestation of pneumonia. Increased
work of breathing accompanied by intercostal, subcostal, and suprasternal retractions,
nasal flaring, and use of accessory muscles is common.
30(8). Sickle cell anemia with acute chest syndrome.
31.(E). in the developed world, cystic fibrosis is the most common cause of clinically
significant bronchiectasis.
32.(A). The most common complaints in patients with bronchiectasis are cough and
production of copious purulent sputum. Younger children may swallow the sputum.
Hemoptysis is seen with some frequency. Fever can occur with infectious
exacerbations. Anorexia and poor weight gain may occur as time passes.
33.(C). CF is responsible for most cases of exocrine pancreatic insufficiency in early life
and is the major cause of severe chronic lung disease in children. It is also responsible
for many cases of salt depletion, nasal polyposis, pansinusitis, rectal prolapse,
pancreatitis, cholelithiasis, and insulin-dependent hyperglycemia. CF may manifest as
failure to thrive and, occasionally, as cirrhosis or other forms of hepatic dysfunction.
34.(A). The earliest pathologic lesion in the lung is that of bronchiolitis (mucous
plugging and an inflammatory response in the walls of the small airways); with time,
mucus accumulation and inflammation extend to the larger airways (bronchitis).
35 (D).
36(8). Failure to thrive 37.5%; persistent respiratory symptoms 45.6%; abnormal stools
28.8%; meconium ileus 19.9%; hepatobiliary disease 1.2%
322
37.(C). More than 95% of males are azoospermic becauseof failure of development of
Wolffian duct structures, but sexual function is generally unimpaired.
38.(E). Dilution, malnutrition, edema, insufficient sweat quantity, hyponatremia, and
cystic fibrosis transmembrane conductance regulator (CFTR)mutations with preserved
sweat duct function are causes of false negative sweat test.
39.(E). The finding of S. aureus or P. aeruginosa on culture of the lower airways
(sputum) strongly suggests a diagnosis of CF. In particular, mucoid forms of P.
aeruginosa are often recovered from CF lungs. B. cepacia recovery also suggests CF. A
wide range of other organisms are frequently recovered, particularly in advanced lung
disease; they include a variety of gram-negative rods, fungi, and nontuberculous
mycobacterial species.
40.(E). Aerosolized antibiotics are often used when the airways are colonized with
Pseudomonas as part of daily therapy. Aerosolized tobramycin, TOBI, used as a
suppressive therapy (on 1 month, off 1 month) may reduce symptoms, improve
pulmonary function, and alleviate the need for hospitalization.
41.(D). Left-right laterality defects are found in PCB; 50% of patients have situs inversus
totalis with transposition of the thoracic and abdominal organs.
42.(D). Heiner syndrome is a cause of diffuse alveolar hemorrhage (DAH) syndromes
without pulmonary capillaritis.
43.(A). Pathologic findings have included elevations of lgE.
44.(C). The anemia of IPH can mimic a hemolytic anemia. Elevations of plasma bilirubin
are caused by absorption and breakdown of hemoglobin in the alveoli.
45.(B). In IPH, early treatment with systemic corticosteroids is the treatment of choice.
46.(A). A commonly encountered risk factor for DVT and PE in the pediatric population
is the presence of a central venous catheter. The presence of a catheter in a vessel
lumen as well as instilled medications can induce endothelial damage and favor
thrombus formation.
47.(D). Metastatic lesions are the most common forms of pulmonary malignancy in
children; primary processes include Wilms tumor, osteogenic sarcoma, and
hepatoblastoma.
48.(C). The most common cause of pleural effusion in children is bacterial pneumonia.
49.(E). Acute asthma is the most common cause of pneumomediastinum in older
children and teenagers. Simultaneous pneumothorax is unusual in these patients.
50.(B). Pneumatocele, lung abscess,and bronchopleural fistula are infectious causes of
pneumothorax in children.
51(E). Hydrothorax is most often associated with cardiac, renal, or hepatic disease. it
can also be a manifestation of severe nutritional edema and hypoalbuminemia. Rarely,
it results from vascular obstruction by neoplasms, enlarged lymph nodes, pulmonary
embolism, or adhesions. It may occur from a ventriculoperitoneal shunt or peritoneal
dialysis and has been reported in congenital parvovirus 319 infection.
51(6). Rupture of an aneurysm is unlikely during childhood.
53.(E).The cells are primarily T lymphocytes.
323
54.(D).An accepteddefinition includes an oxygen requirement for 28 days postnatally,
and the disorder ls graded as mild, moderate or severe on the basis of supplemental
oxygen requirement and gestational age.
55.(A). Physical findings of the pulmonary exam vary with the severity of disease.
Tachypneais a common finding.
56.(C). The etiology of wheezing in BPD may be lower airway inflammation, bronchial
smooth muscle irritation, bronchial smooth muscle hypertrophy, and airway malacia.
57.(B). it may be associated with a connective tissue disorder (Marfan or Ehlers-Danlos
syndrome).
58.(B). Males are affected 4 times more often than females.
S9.(E). Acute respiratory insufficiency is often the most prominent clinical
manifestation of several acute neuromuscular disorders, such as high-level spinal cord
injury, poliomyelitis, Guillain-Barre syndrome, and botulism. Although much more
insidious in its clinical course, respiratory dysfunction constitutes the leading cause of
morbidity and mortality in progressive neuromuscular disorders (e.g., Duchenne
muscular dystrophy, spinal muscular atrophy, congenital myotonic dystrophy,
myasthenia gravis, and Charcot-Marie-Tooth disease).
60.(E). Physical examination reveals a narrowed thorax that, at birth, is much smaller
than the head circumference. The ribs are horizontal, and the child has short
extremities. Chest radiographs demonstrate a bell-shaped chest cage with short,
horizontal, flaring ribs and high clavicles.
6MB). Curves >10 degrees define the presence of scoliosis.
324
- TheCardiovascular
System
QUESTIONS
UHAIR ALMUSAWI
91905?
William syndrome
trisomy 21 (Down syndrome)
X0 (Turner syndrome)
mane?grades l-lll/Vl
louder with patient in supine position
changes with compression ofjugular vein
NP???pleurisy
pneumothorax
pneumonia
325
ofFallot
E. tetralogy
common
MOST cardiac
cyanotic Inthenewborn
topresent
lesion is
period
6.The arteries
ofthegreat
transposition
A. dextroposed
8. leftheartsyndrome
hypopiastlc
C. tetralogyof Fallot
D. truncusarteriosus
E pulmonaryatresia
leftventricular
shows
7.TheECG andasuperior
hypertrophy QRSaxis(between
0' and
-90') in
A. tricuspidatresia
B. Ebsteinanomaly
C. pulmonaryatresia
D. pulmonarystenosls
E. tetralogy of Fallot
9. All the following are causes of heart failure in full-term neonate EXCEPT
asphyxialcardiomyopathy
coarctation of aorta
F'PPP?
hypoplastic left heart
transposition of great arteries
ventricular septal defect
11.TheMOSTcommoncauseof
syncopein childrenis
A. Wolff-Parkinson-White
Syndrome
B. prolongedQT
syndrome
C. atrioventricular block
326
D. neurocardiogenic syncope
E. myocarditis
12.Ofthefollowing,
theMOST
common of theaortain older
signofcoarctation
clinical
children is
A cardiac enlargement
B. notching of the inferior border of the ribs
C. a systolic ejection click or thrill in the suprasternal notch
D. differential blood pressure: arms > legs
E. diminished or absent femoral or lower-extremity pulses
14. The following are causes of congestive heart failure and cardiomegaly during the
newborn period but no murmur EXCEPT
endocardial fibroelastosis
asphyxia
15. SVT in children differ from physiologic sinus tachycardia by all the following EXCEPT
A. sudden onset and termination
8. persistent ventricular rate of >180 bpm
C. fixed RR interval on ECG
D. abnormal P-wave shape or axis or absent P waves
E. evident change in heart rate with activity
327
most
are
factors the
with
associated
strongly of
development
following
17The EXCEPT
disease
Kawasaki
in with
artery
coronary patients
disease
of>16days
offever
A. duration
heartblock
B. first-degree
C. cardiomegaly
D. male gender
E. ageof <1 year
18.The
following side
aremajor EXCEPT
ofPGE1
effects
A. apnea
B. fever
C. cutaneousflushing
D. seizures
E. hypertension
cardiac
ofthefollowing
19.One have
lesions identified
been inmale
more
of the greatarteries
A. transposition
B. atrial septaldefect
C. VSD
D. PDA
E. pulmonicstenosis
physicalnndingis
20. In MOSTpatientswithanASD,the characteristic
A. a rightventricularsystoliclift
B. a fixed splitted 2nd heart sound
C. a systolicejection murmur
D. a short, rumbling mid-diastolic murmur
E. a mild left precordialbulge
328
aortic insufficiency is most often not recognized until late in the lst decade of
life
D. the murmur of a supracristal VSD is usually heard at the mid to upper left
sternal border
E. closure of supracristal ventricular VSDs at the time of diagnosis is not
recommended in an asymptomatic child
23. All the following are signs of coarctation of the aorta EXCEPT
A. femoralpulses are weak or absent
8. bounding pulses of the arms
C. femoral pulse occurs slightly before the radial pulse
D. blood pressure in the legs is lower than that in the arms
E. precordial impulse and heart sounds are usually normal
24. All the following are causes of chronic pulmonary venous hypertension EXCEPT
A. congenital mitral stenosis
B. total anomalous pulmonary venous return with obstruction
C. peripheral pulmonary stenosis
D. left atrial myxomas
E. cor triatriatum
25. Regarding mitral valve prolapse; all the following are true EXCEPT
it is predominantly congenital
it is usually sporadic
26. Sinus bradycardia is due to slow discharge of impulses from the sinus node, the
heart's natural pacemaker. Sinus bradycardia in neonates is considered if sinus rate is
A. <100 beats/min
B. <90 beats/min
C. <80 beats/min
D. <70 beats/min
E. <60 beats/min
329
may
drugs Q-T
long
cause EXCEPT
(LQTS)
syndromes
following
The
28
.A. trimethoprim/sulfamethoxazole
B. erythromycin
C. imipramine
D. pentostam
E. risperidone
MOST
29.The cause
common incompetitive
ofdeath is
athletes
A. aortic stenosis
cardiomyopathy
B. hypertrophic
arteryabnormalities
C. coronary
D. myocarditis
E. longQ-Tsyndrome
(LOTS)
in pediatricpatientsare
agentsfor endocarditis
30.Theleadingcausative
A. group D enterococci
B. viridans-type streptococci
C. Pseudomonas aeruginosa
D. fungal organisms
E. Serratia marcescens
31. The following is a major Duke criterion for the diagnosis of endocarditis
A. new valve regurgitant flow by echocardiography
B. Osler nodes
C. single positive blood culture
D. serologic evidence of infection
E. high erythrocyte sedimentation rate
33. Carefulevaluation
of the characterof t he
physical
pulses earlystepinthe
isanimportant
diagnosisof congenitalheart disease.A with bounding
pulses widepulsepressure
maysuggest thefollowingEXCEPT
A. patent ductus
arteriosus
B. aortic
insufficiency
C.
cardiomyopathy
D. anemia
330
E. anxiety
34. Tall (>25 mm), narrow, and spikedP waves are seen in
A. Ebstein anomaly
B. ventricular septal defect [VSD]
C. patent ductus arteriosus [PDA]
D. severe mitral stenosis
E. mitral regurgitation
and
effusions
35. In uteroheartfailure,oftenwith fetal pleuralandpericardial
generalized ascites (nonimmune hydrops fetalis) may occur in
A. ventricular septal defect
B. coarctation of aorta
C. d-Transposition of great arteries
D. Ebsteinanomaly
E. single ventricle
W909? valvular
coarctation
mitral
aortic
stenosis
stenosis
of the aorta
37. Of the following, the cyanotic cardiac lesion with decreased pulmonary blood flow
is
transposition of the great vessels
single ventricle
99?? syncope
low arterial oxygen saturation
dizziness
331
elevatedliver functiontests
["909
headache
diarrhea
myalgia
40.Postoperative
surgery)
(cardiac bemanaged
can
hypertension With
pulmonary
nitricoxude
andinhaled
A. hyperventilation
B catecholamines
C inhibitors
phosphodiesterase
D. nitroprusside
E. diuretics
@909? anemia
pulmonary hypertension
arrhythmias
44. Pregnancy
iscontraindicated
in motherswith
severe pulmonary hypertension
ventricular septal defects
.MPOF?prosthetic valves
atrial septal defects
unoperated cyanoticcongenital
heart disease
332
45. Surgicalrepair of VSDat the time of
diagnosisshouldbe consideredin
A. small supracristal VSD
B. nonrestrictive VSD
C. hemodynamically significantVSD
D. restrictive VSD
E. VSD with heart failure
46. The MOST common long-term complicationfor those patients who underwent
early repair of complete atrioventricularcanal before the developmentof pulmonary
vascular disease is
A. left AV valve regurgitation
B. subaortic stenosis
C. residual ventricular level shunts
D. complete heart block
E. endocarditis
47. Patients with a small PDA have the following criteria EXCEPT
A. continuous murmur heard best at the left upper sternal border
B. normal peripheral pulses
C. normal pulmonary artery pressure by echocardiography
D. asmptomatic and live a normal life expectancy
E. risk for endocarditis is extremely low
49. Symptoms in patients with aortic stenosis depend on the severity of the
obstruction. Critiml aortic stenosisisassociated with the followire EXCEPT
A. cardiomegaly
B. pulmonary edema
C. weak pulsesin all extremities
D. pale skin
E. maximum intensity of the murmur
50. All the following are true regardingthe diagnosisof coarctationof the com EXCEVI
A. cardiac enlargement are noted with severe coarctation
B. notching of the ribs is common by late childhood
C. doppler is useful for demonstrating the specific site of the obstruction
tools
noninvasive
arevaluable ofcoarctation
forevaluation when
D CTandMRI
isequivocal
theechocardiogram
before
ismandatory
catheterization
E. diagnostic surgery
51.Shone consist
complex EXCEPT
ofthefollowing
A. coarctation of the aorta
B. subvalvar aortic stenosis
C. valvar aortic stenosis
D. pulmonic stenosis
E. mitral stenosis
52.Paroxysmal attacks
hypercyanotic blue, or tet spells)area particular
(hypoxic,
problemduringthe lst 2 yr of life.Theyarecharacterized
by
A. early evening occurrence
8. an increase in intensity of the systolic murmur
C. unpredictable onset
D. metabolic aikalosis
E. more freguent spells in patients with marked cyanosis at rest
334
E. polycythemia
56. D-transposition
of the great vessels,3 commoncyanoticcongenitalanomaly,
accountsfor =5% of all congenital heart disease.In this anomaly, all the following are
true EXCEPT
the systemicveins return to the right atrium
the pulmonary veins return to the left atrium
POP? the connections between the atria and ventricles are normal
the aorta is posterior and to the right of the pulmonary
artery
E. the aorta is anterior and to the right of the pulmonary artery
58. in neonates with total anomalous pulmonary venous return and marked pulmonary
venous obstruction, the chest x-ray demonstrates
A. small heart
B. enlarged heart
C. prominent pulmonary artery
D. prominent right ventricle
E. subaortic stenosis
59. In truncus arteriosus, a single arterial trunk arises from the heart and supplies the
systemic, pulmonary, and coronary circulations. All the following are true EXCEPT
A. VSD is always present
8. the truncus overriding the defect
C. both ventricles are at systemic pressure
D. heart failure ensues immediately after birth
E. clinicalcyanoslsis usuallymild
consists
ofCantrell
62.Pentalogy EXCEPT
ofthefollowing
ectopia cordis
midlinesupraumbilicalabdominaldefect
64. All patients with clinical evidence of a PDA are at increased risk for endocarditis. As
result, PDA should be considered for closure EXCEPT
PDA with severe irreversible pulmonary hypertension
small, hemodynamically insignificant PDA
F1905?
atrial flutter
ventricular tachycardia
digitalis intoxication
W909"?
school performance problems
hepatic toxicity
pulmonary fibrosis
336
67. The following criteria are indicationsfor further investigationof premature
ventricularcontractions
(PVCs) that couldrequiresuppressivetherapyEXCEPT
A. multiform PVCs
B. disappear during exercise
C. R-on-T phenomenon
D. extreme frequency of beats
E. presence of underlying heart disease
68.In urgentsituations
of supraventricular (SVT)
tachycardia of severe
whensymptoms
heart failure have already occurred.
Of the following, the initial management is
A. adenosine by rapid intravenous push
8. DC cardioversion (0.521/kg)
C. verapamil
D. digoxin
E. amiodarone
70. In 90% of cases of endocarditis, the causative agent is recovered from the first 2
blood cultures. Antimicrobial pretreatment of the patient reduces the yield of blood
cultures to
A. 10%
B. 30%
C. 40%
D. 60%
E. 80%
71. Despite the use of antibiotic agents, mortality remains high, in the range of 20-25%.
Serious morbidity occurs in 50-60% of children with documented infective endocarditis.
Of the following, the MOST common morbidity is
A. heart failure
B. pulmonary emboli
C. mycotic aneurysms
D. acquired ventricular septal defect
337
E. heart block
(doxorubicin
cardiotoxicity
72.Anthracycline on rare0CC3$i0n
[AdriamycinD causes
acuteinflammatory
myocardial results
injury,but moreclassically in dilated
inupto30%ofPatients
andoccurs
(DCM)
cardiomyopathy 8W9 a cumulative
doseof
doxorubicin exceeding
A. 250 mg/mZ
B. 350 mg/mZ
C. 450 mg/mZ
D. 550 mg/mZ
E. 650 mg/mZ
74. Diastolic dysfunction and normal systolic function are recognized features of
dilated cardiomyopathy
hypertrophic cardiomyopathy
338
77.Afterload
reducers inchildren
arenotindicated to
withheartfailuresecondary
A. cardiomyopathy
8. severe mitral insufficiency
C. aortic insufficiency
D. ventricular septal defect
E. aortic stenosis
reactions
78.Adverse inhibitors
to angiotensin-converting
enzyme (ACEls) the
include
following EXCEPT
A hypotension
B. hypokalemia
C. maculopapular pruritic rash
D. renal toxicity
E. chronic cough
339
TheCardiovascular
System)
ANSWERS
UHAIR ALMUSAWI m. was. 'w.(_
340
o Transposition of great arteries
0 Total anomalous pulmonary venous return
- Truncus arteriosus
14.(A). Endocardial fibroelastosis is a causeafter the neonatal period.
15.(E). Little change in heart rate with activity, crying, or breath holding.
16.(C).
17.(B). Dysrhythmias (other than firstdegree heart block) is an associated factor.
18.(E). Apnea, fever, cutaneous flushing, seizures, hypotension, and
bradycardia/tachycardia.
19.(A). Gender differences in the occurrence of specific cardiac lesions have been
identified. Transposition of the great arteries and leftsided obstructive lesions are
slightly more common in boys (=65%), whereas atrial septal defect, VSD, PDA, and
puimonic stenosis are more common in girls.
20.(B). In most patients with an ASD, the characteristic finding is that the 2nd heart
sound is widely split and fixed in its splitting during all phases of respiration.
21.(E).
22.(E). Closure of all supracristal ventricular VSDs at the time of diagnosis is commonly
recommended to prevent the development of aortic regurgitation, even in an
asymptomatic child.
23.(C). The radial and femoral pulses should always be palpated simultaneously for the
presence of a radialfemoral delay. Normally, the femoral pulse occurs slightly before
the radial pulse. A radial-femoral delay occurs when blood flow to the descending aorta
is dependent on collaterals, in which case the femoral pulse is felt after the radial pulse.
24.(C). A variety of lesions may give rise to chronic pulmonary venous hypertension,
which when extreme may result in pulmonary arterial hypertension and right-sided
heart failure. These lesions include congenital mitral stenosis, mitral insufhciency, total
anomalous pulmonary venous return with obstruction, left atrial myxomas, cor
triatriatum (stenosis of a common pulmonary vein), individual pulmonary vein stenosis,
and supravalvular mitral rings.
2545). This lesion is not progressive in childhood, and specific therapy is not indicated.
Antibiotic prophylaxis is no longer recommended during surgery and dental
procedures.
26.(B). Sinus rate <90 beats/min in neonates and <60 beats/min in older children is
considered to be sinus bradycardia.
27.(E). Ocular pressme must never be performed, and carotid sinus massage is very
rarely effective.
28.(D). The other antiprotozoal agent, pentamidine isethionate may cause long Q-T
syndrome (LQTS).
29.(B). The most common cause of death in competitive athletes is hypertrophic
cardiomyopathy, with or without obstruction to left ventricular outflow.
30.(B). Viridans-type streptococci (a-hemolytic streptococci) and Staphylococcus
aureus remain the leading causative agents for endocarditis in pediatric patients.
341
31.(A).The Dukecriteriahelp in the diagnosis of endocarditis.Major criteria include
(1) positiveblood cultures(2 separateculturesfor a usual pathogen, 2 or more for less
typicalpathogens),and (2) evidenceof endocarditison echocardiography(intracardiac
mass on a valve or other site, regurgitant flow near a prosthesis, abscess, partial
dehiscenceof prostheticvalves, or new valve regurgitant flow).
32.(C).Lowerlimit of normal is 70/min, average 125/min, and upper limit is 190/min.
33.(C). The presence of diminished pulses in all extremities is associated with
pericardialtamponade, left ventricular outflow obstruction, or cardiomyopathy.
34.(A). Tall (>2.5 mm), narrow, and spiked P waves are indicative of right atrial
enlargement and are seen in congenital pulmonary stenosis, Ebstein anomaly of the
tricuspid valve, tricuspid atresia, and sometimes cor pulmonale. These abnormal waves
are most obvious in leads ll, V3R, and V1. Similar waves are sometimes seen in
thyrotoxicosis. Broad P waves, commonly bifid and sometimes biphasic, are indicative
of left atrial enlargement. They are seen in some patients with large left-to-right shunts
(ventricular septal defect [VSD], patent ductus arteriosus [PDA]) and with severe mitral
stenosisor regurgitation. Flat P waves may be encountered in hyperkalemia.
35.(D). One notable exception is the case of severe regurgitant lesions, most commonly
of the tricuspid valve. In these lesions (e.g., Ebstein anomaly or severe right ventricular
outflow obstruction, the parallel fetal circulation cannot compensate for the volume
load imposed on the right side of the heart. In utero heart failure, often with fetal
pleural and pericardial effusions and generalized ascites (nonimmune hydrops fetalis)
may occur.
36.(B). The most common lesions in this group are those that cause Left-to~right
shunting: atrial septal defect, ventricular septal defect (VSD), AV septal defects (AV
canal), and patent ductus arteriosus.
37.(D). This group of congenital heart lesions include (tetralogy of Fallot, pulmonary
atresia with an intact septum, tricuspid atresia, total anomalous pulmonary venous
return with obstruction).
38.(D). Arterial oxygen saturation is usually normal unless there is an associated
intracardiac shunt.
39.(B). Flushing, headache, diarrhea, hypotension, fluid retention, exacerbation of
heart failure, anemia, elevated liver function tests, and palpitations are common side
effects of bosentan.
40.(A).
41.(B). Patients who have undergone surgery entailing the use of cardiopulmonary
bypass, eSpecially in the newborn period, should be watched carefully during their
early school years for signs of mild to moderate learning disabilities, which are often
amenable to early remedial intervention. The risk is higher in patients who have
undergone repair using hypothermic total circulatory arrest than in those where
systemicblood flow is maintained usingcardiopulmonary bypass.
42.(E). The postpericardiotomy syndrome may occur toward the end of the lst
postoperativeweek or may sometimesbe delayed until weeks or months after surgery-
342
This febrile illness is characterized by fever, decreased appetite, listlessness, nausea,
and vomiting. Chest pain is not always present, so a high index of suspicion should be
maintained in any recently postoperative patient.
43.(E). Many argue that only for isolated patent ductus arteriosus is total repair really
achieved, with no requirement for long-term follow-up.
44.(A). Pregnancy is contraindicated in mothers with severe pulmonary hypertension,
severe obstructive lesions, and Marfan syndrome, aortic root >40 mm.
45.(A). The exception to this rule is patients with small supracristal or perimembranous
VSD with associated prolapse of the aortic cusp into the defect resulting in progressive
aortic regurgitation. These patients should be considered for surgical repair at the time
of diagnosis to prevent progressive aortic valve damage.
46.(A). Overall, for those patients who underwent early repair before the development
of pulmonary vascular disease, the long-term prognosis is good. The most common
long-term complication is left AV valve regurgitation, with approximately 5-10% of
patients requiring surgical revision for left AV valve repair or replacement during
followup.
47.(E). A silent PDA is a tiny defect that cannot be heard by auscultation and is only
detected by other nonclinical means such as echocardiography. Life expectancy is
always normal in this population and the risk for endocarditis is extremely low. Patients
with a small PDA have an audible long-ejection or continuous murmur heard best at the
left upper sternal border that radiates to the back. In addition, they have normal
peripheral pulses. Because there is negligible left to right shunting these patients have
normal left aorta (LA) and left ventricle (LV) size and normal pulmonary artery pressure
by echocardiography and chest x-ray. These patients like those with silent PDAs are
aysmptomatic and live a normal life expectancy. They have a higher risk for
endocarditis.
48.(C). For those patients with severe stenosis (defined as a peak gradient of
>50 mm Hg), the majority ultimately require an intervention, either surgery or balloon
valvuloplasty by age 25 yr.
49.(E). If cardiac output is significantly decreased, the intensity of the murmur at the
right upper sternal border may be minimal.
SO.(E). In cases that are well defined by echocardiography, CT, or MRI, diagnostic
catheterization is not usually required before surgery.
51.(D). Congenital mitral stenosis is a rare anomaly that can be isolated or associated
with other defects, the most common being subvalvar and valvar aortic stenosis and
coarctation of the aorta (Shone complex).
52.(C). The spells occur most frequently in the morning on initially awakening or after
episodes of vigorous crying. Temporary disappearance or a decrease in intensity of the
Systolic murmur is usual as flow across the right ventricular outflow tract diminishes.
The onset is usually spontaneous and unpredictable. Spells are associated with
reduction of an already compromised pulmonary blood flow, which, when prolonged,
results in severe systemic hypoxia and metabolic acidosis. Infants who are only mildly
343
cyanotic at rest are often more prone to the development of hypoxic spells because
they have not acquired the homeostatic mechanisms to tolerate rapid lowering of
arterial oxygen saturation, such as polycythemia.
53.(D). Because metabolic acidosis develops when arterial P02 is <40 mm Hg, rapid
correction (within several minutes) with intravenous administration of sodium
bicarbonate is necessary if the spell is unusually severe and the child shows a lack of
response to the foregoing therapy.
54 (C). Concavity in the region of the main pulmonary artery.
55.(B). The severity of symptoms and the degree of cyanosis are highly variable and
depend on the extent of displacement of the tricuspid valve and the severity of right
ventricular outflow tract obstruction.
56.(D). In normally related great vessels, the aorta is posterior and to the right of the
pulmonary artery; in d-transposition of the great arteries (d-TGA), the aorta is anterior
and to the right of the pulmonary artery (the d indicates a dextropositioned aorta,
transposition indicates that it arises from the anterior right ventricle).
S7.(A). Obstructed TAPVR is a pediatric cardiac surgical emergency because
prostaglandin therapy is usually not effective.
58.(A). ln neonates with marked pulmonary venous obstruction, the chest x-ray
demonstrates a very dramatic perihilar pattern of pulmonary edema and a small heart.
59.(D). When pulmonary vascular resistance is relatively highimmediately after birth,
pulmonary blood flow may be normal; as pulmonary resistance drops in the lst mo of
life, blood flow to the lungs is greatly increased and heart failure ensues.
60.(D). Although cyanosis may not always be obvious in the lst 48 hr of life, a grayish-
blue color of the skin is soon apparent and denotes a mix of cyanosis and poor
perfusion. All of the peripheral pulses may be weak or absent. This lesion may be
isolated or associated in 5-15% of patients with known genetic syndromes, such as
Turner syndrome, trisomy 13, 18, or 21, Jacobsen syndrome (llq deletion), Holt-Dram
syndrome, andRubinstein-Taybi syndrome. Occasionally it is familial and inherited as an
autosomal recessive trait.
61.(C). All other options are features of asplenia (right isomerism).
62.(D). Defect of the lower sternum.
63.(A). Idiopathic or familial disease is the most common in pediatric patients (55%).
followed by pulmonary hypertension secondary to congenital heart disease (35%) and
chronic respiratory disorders (15%).
64.(A). All PDAsexcept for small silent PDAs and those patients with severe irreversible
pulmonary hypertension should be considered for closure.
6S.(E). Other options are treated by procainamide.
66.(C). Other options are side effects of amiodarone.
67.(B). It is important to distinguish PVCsthat are benign from those that are likely to
lead to more severe arrhythmias. The former usually disappear during the tachycardia
of exercise. If they persist or become more frequent during exercise, the arrhythmia
may have greater signihcance.
68.(B). In urgent situations when symptoms of severe heart failure have already
occurred, synchronized DC cardioversion (0.5-2 J/kg) is recommended as the initial
management.
69.(C).
70.(D). Antimicrobial pretreatment of the patient reduces the yield of blood cultures to
50-60%. The microbiology laboratoryshould be notified if the patient has received
antibiotics so thatmore sophisticated methods can be used to recover the offending
agent.
71.(A). The most common is heart failure caused by vegetations involving the aortic or
mitral valve. Myocardial abscesses and toxic myocarditis may also lead to heart failure
without characteristic changes in auscultatory findings and, occasionally, to life~
threatening arrhythmias.
72.(D). Exceeding 550 mg/m2. The risk of toxicity appears to be exacerbated by
concomitant radiation therapy.
73.(D). Dramatic atrial dilation can occur as a result of the abnormal myocardial
compliance and high ventricular diastolic pressure.
74.(D).
75.(C). Coxsackievirus and other enteroviruses, adenovirus, parvovirus, Epstein-Barr
virus, parechovirus, influenza virus, and cytomegalovirus are the most common
causative agents in children, though most known viral agents have been reported. In
Asia, hepatitis C virus appears to be significant as well.
76.(B). Rhabdomyomas are the most common pediatric cardiac tumors and are
associated with tuberous sclerosis in 7095% of cases.
77.(E). ACEls and ARBs are not generally used in the presence of stenotic lesions of the
left ventricular outflow tract because of concern over coronary perstion.
78.(B). Adverse reactions to ACEIs include hypotension and its sequelae (weakness,
dizziness, syncope) and hyperkalemia.
79.(C). The National High Blood Pressure Education Program Working Group on High
Blood Pressure in Children and Adolescents published the Fourth Report on the
Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and
Adolescents (Fourth Report) in 2004.
The Fourth Report defined hypertension as average systolic blood pressure (SBP)
and/or diastolic BP that is 295th percentile for age, sex, and height on 23 occasions.
Prehypertension was defined as average SBP or diastolic BP that are 290th percentile
but <95th percentile.
80.(C). Other options are responsible for acute/ intermittent hypertension.
81.(A). Measuring serum potassium is essential because hypokalemia may be present
in Liddle syndrome, glucocorticoidremedial aldosteronism, and apparent
mineralcorticoid excesssyndrome, while hyperkalemia may be. seen in Gordon
Syndrome.
345
of the Blood
Diseases
QUESTIONS
WA!
ofcongenital
features
1.Thehematologic anemia
hypoplastic (Diamond-Biackfan
include
Anemia) EXCEPT
allthefollowing
A. normocyticanemia
B. reticulocytopenia
ofredbloodcellbonemarrowprecursors
orabsence
C. deficiency
D. elevatedfetal hemoglobin
(i-lbF)
E. elevated serum iron levels
2.TheMOST
specific todifferentiate
testthatishelpful anemia
Diamond-Blackfan from
of childhoodis
transienterythroblastopenia
A. hemoglobin electrophoresis
B. reticulocytes count
deaminase(ADA)enzymelevelassay
C. erythrocyteadenosine
D. bone marrow examination
E. mean corpuscularvolume (MCV)
91,009"?
antithymocyte globulin (ATG)
fully matched-relatedstem cell transplantation
fully matched~unreiatedstem cell transplantation
aretrueregarding
Transient
following (TEC)
ofchildhood
Erythroblastopenla
2x22???
A. it ismorecommonthan congenitalhypopiastic anemia
(Diamond-Biackfan)
B. most of the affected children are older than 12 mo at onset
C. Parvovirus 819 infections is a common causative agent
D. Virtually all children recover within 12 months
E. corticosteroid therapy is of no value
7. All the following are true regarding physiologic anemia of infancy EXCEPT
A. it is exaggerated in premature infants
B. the hemoglobin concentration range is between 9-11 g/dL
C. the hemoglobin concentration rarely falls below 10 g/dL in healthy infants
D. vitamin E deficiency does not play a role
E. iron deficiency is a contributing factor
mom»?
an infant fed on pasteurized cow's milk
a child with chronic use of phenytoin anticonvulsant
a child with trimethoprim prophylaxis for recurrent urinary tract infection
9. A 1-year-old child has folic acid deficiency since the age of 4 months, the best
indicator of this deficiency is
A. significant increment of mean corpuscularvolume MCV
8. significant fall of reticulocytes count
C. high level of lactate dehydrogenase LDH
D. decrease level of RBCfolate
E. increase number of hypersegmented neutrophils
347
iron deficiencyanemia
anemia
megaloblastic
9153.09?
sideroblastic anemia
thalassemia
sickle cell anemia
12.Thelaboratory withthediagnosis
likelyconsistent
thatis MOST
finding of
spherocytosis is
countof morethan10%
A. highreticulocytes
B. normalmeancorpuscularvolumeMCV
C. low meancorpuscularhemoglobinMCH
D. highmeancorpuscular
hemoglobin MCHC
concentration
E. indirect hyperbilirubinemia
348
C. nephroticsyndrome
D. hematuria
E. acute renal injury
18. Blood transfusion therapy in a child with sickle cell anemia is indicated in all the
following conditions EXCEPT
acute chest syndrome
stroke
19. The best assessment of iron overload for patients with thalassemia major is
achieved by
A. liver MRI
B bone marrow biopsy
C. serum iron
D. serum ferritin
E. total iron binding capacity
20. The criteria for successful bone marrow transplantation in thalassemic patients
include all the following EXCEPT
ageyoungerthan 15-year-old
no hepatomegaly
349
.
D. localradiotherapy_ : ~ o
E. vitaminDsupplementation
.
major; . . .
B-thalassemia . heisonchronic
old chil d-with
22. Youare evaluatin8 a 4-Y83" suspect transfusuon-Induced
0f 1 year; you
transfusiontherapysincethe age
hemosiderosis. .
LEAST
is
that tobe
likely byiron
affected
°
atthis
deposition
Ofthe the
organ
following, J
time is - . .
~
A. liver
8. pancreas
C. heart
D. pituitary gland
E. thyroid gland
5-moold
23.Ahealthy pale.
boyappears Labfindings
isunremarkable.
Examination
Hb8.1g/dl;WBC
include: platelets
4,800/mm3; MCV,
144,000/mm3; film
111fl; blood
showedhypersegmented serum812,65 pg/mL(low).The infant is
neutrophils;
vigorously breast fed.
Of the following, the NEXTstep of the management is
performing Schilling test _
check serum 812 of the mother
'
25. Hereditary intrinsic factor deficiency (HIFD),formerl - --
differs
anemia, from
the adult
typical anemia
pernicious
b::"lfh: pernICIous
:zllgemtal
A. it isprominent
ataround
3moof EXCEPT
Wing
age
B. stomach secretes acid normally
C. stomach is histologically normal
D. there are no antibodies to parietal cells
E. there are no associated endocrine disorders
26. Increased
incubated
osmotic of
bytheaddition
fragilitytestthatisnotcorrected
glucose is MOST likely suggestive of
A. hereditary spherocytosis
B. hereditary elliptocytosis
C. hereditary stomatocytosis
D. glucose-6phosphate dehydrogenase deficiency
E. pyruvate kinase deficiency
29. Fragmentation hemolysis by mechanical injury may be seen in all the following
EXCEPT
A. extensive burns
B. Kasabach-Merritt syndrome
C. after cardiac surgery for prosthetic heart valve replacement
D. thrombotic thrombocytopenic purpura (TIP)
E. hemolytic uremic syndrome (HUS)
30. All the following conditions may be associated with iron dehciency EXCEPT
A. hookworm infestation
B. prolonged intravascuiar hemolysis
C. celiac disease
0. congenital heart disease with right to ieft shunt
E. prolongeduse of isoniazid(INH)
351
E. factor XI
42.Inwhich
ofthefollowing youshould
conditions thatisfrozen
useaplasma within
24 hours after collection?
A. factor V deficiency
B. factor VII deficiency
C. factor IX deficiency
D. factor X deficiency
E. factor XI deficiency
44. In severe liver disease with prolongation of the bleeding time, the best effective
treatment before doing liver biopsy is
A. intravenous vitamin K
B. intramuscularvitaminK
C. oral vitamin K
D. fresh frozen plasma
E. intravenous DDAVP
female
46.A 16-year-old isfoundto havemultiple andhypochromic
bruising microcytic
anemia; she
duringherfirstpregnancy noticed ofthebruises;
disappearance shehasa
past of
history emergency
uncomplicated appendectomyatthe of 10
age year.
thedrugMOST
Ofthefollowing, likelybeneficial
foralleviation
ofhersymptoms
is
ferrous sulphate
acetate(DDAVP)
desmopressin
GDP?
heterozygous individuals have less risk for thrombosis than homozygotes
heterozygotes have an. increased risk of arterial thrombosis
. there is an increased frequency of thrombosis while receiving oral
contraceptive agents
E. there is an increased risk of recurrent abortions
.MPDPP
renal infarction
myocardial infarction
pulmonary embolism
W905?
prothrombin time (PT)
thrombin time (1T)
clot solubility test
k.*~ 91.0090?
it should not be given fora child with bacterial endocarditis
it can be given intramuscularly
it needs monitoring by PTl' test
52. All the following are true regarding low molecular weight heparin (LMWH) in
comparisonwith unfractionatedheparin (UFH) EXCEPT
A. it is more stable
B. it is ease to titrate
C. it is monitored by activated PTT
D. it can be used subcutaneously
E. it can be used as an outpatient
S3. A healthy 5-dayold male neonate develops bruising and melena. The pregnancy,
and delivery course were unremarkable. The mother received heparin therapy for
recurrent abortions secondary to lupus anticoagulants. The infant is vigorously
breastfeeding. Examination reveals only multiple bruises. Lab tests reveals:
hemoglobin, 8.4 g/dL; WBC, 8600/mm3; platelets count, 200000/mm3; PTT, 74
seconds; PT, 28 seconds; serum fibrinogen is normal.
Of the following, the MOST likely cause of this bleeding is
A. disseminated intravascular coagulopathy (DIC)
B. hemophilia
C. vitamin K deficiency
D. heparin therapy
E. lupus anticoagulants
54. A previously healthy 10-month-old boy develops pallor over 2 weeks. Examination
is unremarkable except for pallor. Lab tests includes: hemoglobin, 4.9 g/dL; WBC,
6700/mm3; platelets count, 180000/mm3; MCV, 79 fL; reticulocyte count 0.8%;
hemoglobin electrophoresis, normal.
Of the following, the MOST likely cause for these findings is
A. pure red cell aplasia
B. transient erythroblastopenia of childhood
C. thalassemia minor
D. aplastic anemia
E. parvovirus infection
55. You are evaluating a 5-year-old male child with multiple ecchymoses distributed all
over the extremities and trunk; examination reveal a palpable spleen 3 cm below the
left costal margin. Lab findings include: hemoglobin, 11.3 g/dL; white blood cell count,
8700/mm3; platelets count, 21000/mm3.
Of the following, the most proper NEXT step is
A. bleeding time
B. coagulation profile
C. platelets aggregation test
D. abdominal ultrasonography
E. bone marrow study
59. Which of the following drugs is least likely to cause thrombocytopenia in children?
A. valproicacid
B. phenytoin
C. sulfonamides
mvsu--
D. trimethoprim-sulfamethoxazole
LA
E. heparin
k.-
B. Bernard-Soulier syndrome
C. Wiskott-Aldrich syndrome (WAS)
D. Glanzmann's Thrombasthenia
E. type 28 von Willebrand disease
M...
61. Thrombocytopenia that improves with age is MOST likely a feature of
congenital amegakaryocytic thrombocytopenia
Bernard-Soulier syndrome
357
62. You are going to explain the role of anti-D therapy to the parents of a 4-year-old
boy with acute immune thrombocytOpenia (lTP) who has several ecchymotic lesions
located over extremities and trunk. There is no mucosal bleeding.
An important statement that should be included during the discussion is
A. anti-D therapy is more effective than IVIG therapy in acute ITP
8. anti-D therapy is very effective to all children with lTP
C. when anti-D therapy is given, monitoring of hemoglobin concentration is
recommended
0. anti-D therapy is used in acute lTP only
E. anti-D therapy can be used as initial treatment for children with acute lTP
63. A 9-month-old boy appears pale. Examination reveals a palpable liver 3 cm below
the right costal margin and palpable spleen 4 cm below the left costal margin. Lab
findings include: hemoglobin concentration, 6.4 g/dl; mean corpuscular volume (MCV),
67 fL; and WBC, 15,000/mm3.
0f the following, The MOST valuable test to confirm the diagnosis is
blood smear
serum iron, total iron binding capacity, and serum ferritin
WPFF?hemoglobin electrophoresis
bone marrow
abdominal
study
ultrasonography
64. You are explaining the risk of sepsis after splenectomy to medical students. An
important statement that should be included during this discussion is that risk is
especially high in children
older than 5 yr at the time of surgery
with hereditary spherocytosis
65. You are evaluating a 7-year-old male child with cervical Iymphadenopathy; the
MOST likely finding that raise your suspicion of malignancy is
A. matted nodes
B. fluctuant nodes
C. fixed nodes
D. presence of systemic symptoms
E. no regression in the size of the nodes to its normal within 2 weeks
66. Of the following, the MOST accurate reflection of active erythropoiesis of the bone
marrow is
A. hemoglobin concentration
358
B. packed cell volume (PCV)
C. reticulocyte count
D. mean corpuscular volume (MCV)
E. reticulocyte index
67. Hematologic disease with upper limb orthopedic congenital abnormalities with
presence of thumbs is MOST likely seen in
A. Fanconi anemia
B. thrombocytopenia with Absent Radii (TAR) Syndrome
C. congenital Amegakaryocytic Thrombocytopenia
D. Glanzmann Thrombasthenia
E. Bernard-Soulier Syndrome
69. A 5~year-old child has multiple bruises on her extremities and oral mucosal bleeding
of 3 days duration; she had a mild respiratory tract infection 2 weeks before; physical
examination was normal apart from multiple ecchymoses and petechiae.
Of the following, the NEXT diagnostic step is
A. complete blood count
B. prothrombin time
C. bleeding time
D. partial thromboplastin time
E. antinuclear antibody titer
359
E. hemolyticuremic syndrome (HUS)
73. 0f the following, the LEASTlikely feature of acute immune thrombocytopenia (ITP)
is
A. a preceding viral infection
B. isolated thrombocytopenia
C. mucocutaneous bleeding
D. isolated splenomegaly
E. increased number of megakaryocytes in the bone marrow
74. A 3-year-old male child develops hematoma and bruising of his right hand next day
after falling on the ground; the mother stated that her child has a poor wound healing
and a history of delayed umbilical separation during the neonatal period.
Of the following, the MOST valuable test for this case is
A. bleeding time
8. partial thromboplastin time
C. prothrombin time
D. thrombin time
E. clot solubility test
75. A 5-year-old child, with hemophilia A of severe type, presents to the emergency
unit with a groin pain after a minor trauma to his back; his blood pressure is 60/30 mm
Hg; his pulse rate is 180/min; he holds his right hip in a flexion position with internal
rotation.
0f the following, the NEXT step in the management of this child is
A. factor Vlll replacement therapy
B. intravenous 1-deamino-8-d~arginine vasopressin (DDAVP)
C. factor VIII assay
D. abdominal ultrasonography
E. abdominal computed tomography (CT) scan
76. In a child with a recent diagnosis of hemophilia A who developed moderate
epistaxis, the aim of the treatment is to increase the level of factor VIII to
A 5-20%
B. 35-50%
C. 55-60%
D. 65-70%
E. 80-100%
77. Allogeneic bone marrow stem cell transplantation is least likely to be required in
Fanconi anemia
wpnw? Glanzmanns
Bernard-Soulier
Wiskott-Aldrich
91.00.05?
corticosteroids
platelets transfusion
no treatment
80. The standard care for MOST children with severe hemophilia is
A. prevention by F Vlll replacement therapy to prevent spontaneous bleeding and
early joint deformities
aggressive treatment by F Vlll replacement therapy when significant bleeding
occurs
avoid trauma
361
Diseasesof the Blood
ANSWERS
@SAMAAUUMAILY
1.(A). The RBCsin Diamond-Blackfananemiaare usually macrocytic for age, but there is
no hypersegmentation of neutrophils.
2.(C). Erythrocyte adenosine deaminase (ADA) activity is increased in most patients
with congenital hypoplastic anemia, a finding that helps distinguish this disorder from
acquired transient erythroblastopenia of childhood. Also, because elevated ADA
activity is not a fetal RBCfeature, measurement of this enzyme is helpful in diagnosing
DiamondBlackfan anemia in very young infants. Hemoglobin F may be increased in
20% of cases with transient erythroblastopenia of childhood at the time of diagnosis.
3.(C). The anemia of hemolytic disease of the newborn can have a protracted course,
but this usually terminates spontaneously at 5-8 wk of age. Aplastic anemic crises,
frequently caused by parvovirus 819 infections, may complicate various types of
chronic hemolytic disease, but usually after the first several months of life.
4.(A). Corticosteroid therapy is beneficial in three fourths of patients; the mechanism
of its effect is unknown. Stem cell transplantation from a related histocompatible
donor has a role in children who do not respond to corticosteroids and who have
demonstrated a several-year need for RBC transfusions; The survival results for
matched-related donors have been very encouraging, but the responses have been
much inferior with the use of partially mismatched siblings or matched unrelated
donors.
5.(C). in rare instances, a prolonged case of apparent TEC may be caused by parvovirus
induced RBC aplasia, occurring in children with hemolytic anemia or congenital or
acquired immunodeficiencies.
6.(B). The serum iron level in anemia of chronic disorder is low, but without the
increase in total iron-binding capacity (serum transferrin) that occurs in iron deficiency.
This pattern of low serum iron and low to normal iron-binding protein (serum
transferrin) is a regular and valuable diagnostic feature.
7.(E). The iron previously stored from the degraded fetal RBCs can be used for
hemoglobin synthesis. The supply of stored iron is sufficient for hemoglobin synthesis,
even in the absence of dietary iron intake, until approximately 20 wk of age. Unless
there has been significant blood loss, iron stores should be sufficient to maintain
erythropoiesis early on.
8.(C). Goats milk and powdered milk are deficient with folic acid. Human breast milk,
pasteurized cow's milk and infant formulas provide adequate amounts of folic acid.
Anticonvulsants may decrease folate absorption. Trimethoprim may affect folate
metabolism.
9.(D).
362
10.(A). In progressive iron deficiency, a sequence of biochemical and hematologic
events occurs. First, the tissue iron stores represented by bone marrow hemosiderin
disappear.
11.(C). RDW is usually high in IDA, megaloblastic anemia, and sideroblastic anemia, but
it is extremely high in the last.
12.(D). The MCHC is a measure of cellular hydration status. A high value (3638 g/ dL) is
characteristic of spherocytosis and a low value is commonly associated with iron
deficiency.
13.(A). After splenectomy, osmotic fragility often improves because of diminished
splenic conditioning and less RBC membrane loss, but not resolved.
14.(C). Hemolytic anemia may be associated with hereditary stomatocytosis, but
splenectomy is not a recommended treatment. Persistent symptomatic thrombocytosis
may follow splenectomy if the hemolysis is not eliminated or markedly decreased.
Patients have developed a life-threatening tendency toward in situ thrombosis after
splenectomy in association with the abnormal adherence of the stomatocytic RBC to
vascular endothelium in conjunction with the thrombocytosis.
15.(D). Flow cytometry is the best diagnostic test for PNH. With the use of anti-C059
for RBCs and anti-C055 and anti-C059 for granulocytes, flow cytometry is more
sensitive than the classic RBC lysis tests in detecting the reduced glycolipid-bound
membrane proteins.
16.(E). Renal function is progressively impaired by diffuse glomerular and tubular
fibIOSiS leading to chronic, rather than acute, renal shutdown.
17.(D). There is an increased incidence of urinary tract infection with bacteremia in
young women with sickle cell anemia.
18.(D). Blood transfusions usually do not reverse ischemic damage. Even while on
chronic transfusion therapy children can still have transient cerebral ischemia as well as
avascular necrosis of the hips and shoulders.
19.(A). Quantitative liver iron by approved MRl technology is the best indicator of total
body iron stores and should be obtained in chronically transfused patients after chronic
transfusion therapy has initiated.
20.(C).
21.(D). Extramedullary hematopoiesis can occur in the vertebral canal, compressing the
spinal cord and causing neurologic symptoms; the latter is a medical emergency
requiring immediate local radiation therapy to halt erythropoiesis.
22.(C). Iron is initially deposited in the liver. Liver hemosiderosis develops after 1 yr of
chronic transfusion therapy and is followed by iron deposition in the endocrine system.
This leads to a high rateof hypothyroidism, hypogonadotropic gonadism, growth
hormone deficiency, hypoparathyroidism, and diabetes mellitus. After 10 yr of
transfusion, cardiac dysfunction secondary to hemosiderosis begins.
23.(B). Older children and adults have sufficient vitamin 812 stores to last 3-5 yr.
However, in young infants born to mothers with low vitamin 312 stores (breast fed
363
infants of vegans mothers or having pernicious anemia), clinical signs of cobalamin
deficiency can become apparent in the first 4-5 mo of life.
24.(D). The o-thalassemia traits present as a microcytic anemia that can be mistaken
for iron~deficiency anemia. The hemoglobin electrophoresis is normal. The diagnosis is
confirmed by DNA testing.
25.(A). In HIFD, symptoms become prominent at an early age (6-24 mo), consistent
with exhaustion of vitamin 312 stores acquired in utero.
26.(E). Autohemolysis is moderately or markedly increased in pyruvate kinase
deficiency, but the addition of glucose does not regularly correct the abnormality as it
does in hereditary spherocytosis.
27.(E). Penicillin may cause hemolysis via the "hapten" mechanism (immune but not
autoimmune) by binding tightly to the RBC membrane. Antibodies to the drug, either
newly or previously formed, bind to the drug molecules on RBCs, mediating their
destruction in the spleen.
28.(E). Splenectomy is not useful in cold agglutinin disease.
29.(A). Extensive burns may directly damage the RBCs and result in hemolysis that
results in the formation of spherocytes. Blood loss and marrow suppression may
contribute to anemia and require blood transfusion; others cause RBC destruction by
mechanical injury.
30.(E). Secondary iron deficiency may complicate the intravascular hemolysis because
of urinary hemoglobin and hemosiderin iron loss. Cardiovascular defects involving
right-to-Ieft shunts interfere with proper oxygenation leading to secondary
polycythemia which may cause iron deficiency due to increased RBC demands
production. INH therapy may cause secondary sideroblastic anemia with high serum
iron and increased transferrin saturation.
31.(B). Parvovirus 819 is classically associated with isolated red cell aplasia, but in
patients with sickle cell disease or immunodeficiencies it can result in transient
pancytopenia.
32.(E). Transfusion of FFP is no longer recommended for treatment of patients with
severe hemophilia A or B or for factor VII deficiency because safer factor VIII, Ix, and VII
concentrates are available; also it is not indicated for correction of hypovolemia or as
immunoglobulin replacement therapy, because safer alternatives exist; An important
use of FFP, albeit rare in children, is for rapid reversal of warfarin effects in patients
who are actively bleeding or who require emergency surgery (i.e., in whom functional
deficiencies of factors lI, VII, IX, and X cannot be rapidly reversed by vitamin K).
33.(D). Transfusion of FFP is efficacious for the treatment of deficiencies of clotting
factors II, V, X, and XI. Factor XIII and fibrinogen deficiencies are treated with
cryoprecipitate. FFP also contains several anticoagulant proteins (antithrombin III,
protein C, and protein S), whose deficiencies have been associated with thrombosis.
34.(D). In neonates, Clotting times are prolonged, owing to developmental deficiency
of clotting proteins, and FFP should be transfused only after reference to normal values
expected for the birthweight and age of the infant. The use of prophylactic FFP
364
transfusions to prevent intraventricular hemorrhage in premature infants is not
recommended.
35.(C).
36.(E). CMV infections may be transmitted by leukocyte containing blood component
transfusions; FFP does not transmit such infection because it is acellular component.
37.(E). Unlike the thrombin time, the reptilase time is not sensitive to heparin and is
prolonged only by reduced or dysfunctional fibrinogen and fibrin split products.
38.(B).
39.(D). Deficiencies of the contact factors prolong the PTT but are not causes of clinical
bleeding. These factors include factor XII, prekallikrein, and high molecular weight
kininogen.
40.(A).
41.(D). Systemic amyloidosis may be associated with factor X deficiency owing to the
adsorption of factor X on the amyloid protein. In the setting of amyloidosis, transfusion
therapy is often not successful because of the rapid clearance of factor X.
42.(A). Factor V is lost rapidly from FFP. It is therefore important to use FFP that is less
than 2 mo old. Because the plasma halflife of factor VII is 2-4 hr, therapy with FFP is
difficult and often complicated by fluid overload.
43.(B). The usual screening tests for hemostasis are normal in patients with factor XIII
deficiency, antiplasmin deficiency, and plasminogen activator inhibitor deficiency.
44.(E). DDAVP (0.3 ug/kg IV) has been found to be effective in shortening the bleeding
time and has been used effectively to augment hemostasis prior to liver biopsy.
45.(A). Vitamin K-dependent clotting factors include factors II, VII, IX, X, protein C, and
protein S.
46.(B). Von Willebrand disease (VWD) is the most likely cause for this case;
menstruating female may present with iron deficiency due to menorrhagia. Because
VWF is an acute-phase protein, stress will increase its level; thus, patients may not
bleed during appendectomy and childbirth. Bruising symptoms may diminish during
pregnancy, because the VWF levels may double or triple during pregnancy.
47.(B). Type 2N VWD is caused by the reduction of factor VIII binding by VWF. This
disorder has also been termed autosomal hemophilia. With this variant, platelet
interaction with VWF is normal but the 2N VWF binds weakly (or not at all) to factor
VIII, resulting in rapid clearance of factor VIII that is weakly complexed to VWF.
48.(C). Individuals who are heterozygous have a 5-7fold increase in risk of venous
thrombosis
49.(E). Pulmonary embolism is a feature of venous thrombosis.
50(8). The lupus anticoagulant causes a prolongation of the PTT that fails to correct on
1:1 mixing with normal plasma. The antibody is directed against the phospholipid used
as a reagent in the PTT.
51.(D).
52.(C). Unlike UFH, which is monitored using the aPTT, LMWH is monitored via the anti-
Xa activity.
365
53.(C). Heparin does not cross the placenta, and so it does not affect the baby. DIC
causes prolonged PTT, PT, thrombocytopenia and low serum fibrinogen. Hemophilia
causes prolonged PTT only. Lupus anticoagulants may cause thrombosis rather than
bleeding and usually associated with prolonged PTT because of the presence of
antiphospholipids that affect the phospholipid reagent.
54.(B). Normal hemoglobin electrophoresis usually excludes thalassemia and Diamond~
Blackfan syndrome. Anemia due to parvovirus infection usually occurs in children with
hemolytic anemia or immunodeficiency. In aplastic anemia, the anemia is usually
macrocytic rather than normocytic.
55.(E). Although spleen may be palpable in 10% of children with lTP, bone marrow
examination is mandatory when there is an unusual presentation.
56.(8). In Bernard-Soulier syndrome there is moderate thrombocytopenia and
abnormal ristocetin test.
S7.(E).
58.(A). In 70-80% of children who present with acute lTP, spontaneous resolution of
their lTP will occur within 6 mo.
59.(E). Common drugs used in pediatrics that cause thrombocytopenia include valproic
acid, phenytoin, sulfonamides, and trimethoprim-sulfamethoxazole. Heparin-induced
thrombocytopenia is seldom seen in pediatrics.
60.(C). WAS is characterized by thrombocytopenia with tiny platelets, eczema, and
recurrent infections due to immune deficiency.
61(0); Thrombocytopenia of TAR syndrome frequently remits over the first few years
oflife.
62.(C). The role of IV antiD in initial therapy of acute lTP is under investigation; the
response appears to be somewhat slower than after IVIG; it is effective for Rh+
children; it can be used in acute or chronic lTP.
63.(C). l3=thalassemia major is the most likely diagnosis; hemoglobin electrophoresis is
quite helpful to confirm the diagnosis. Blood smear usually shows hypochromic
microcytic RBCs but it is difficult to differentiate between iron deficiency anemia and
thalassemia.
64.(E). This risk of sepsis is especially high in children younger than 5 yr at the time of
surgery. The risk of sepsis is less after splenectomies performed for trauma, RBC
membrane defects, and immune thrombocytopenia than when there is pre-existing
immune deficiency (Wiskott-Aldrich syndrome, Hodgkin disease) or reticuloendothelial
blockade (storage diseases, severe hemolytic anemias).
65.(C). A firm fixed node to the surrounding tissue should always raise the question of
malignancy, regardless of the presence or absence of systemic symptoms or other
abnormal physical findings. Fluctuance suggests abscess formation. Tuberculous nodes
may be matted.
66.(E). The Reticulocyte production index (RPl, also called a corrected reticulocyte
count) is a calculated value used in the diagnosis of anemia. This calculation is
366
necessary because the raw reticulocyte count is misleading in anemic patients.
Reticulocyte index = reticulocyte countx (observed hematocrit/normal hematocrit).
67.(B). TAR is characterized by absent radii with presence of thumbs; this distinguishes
from Fanconi anemia in which thumbs are absent.
68.(E).
69.(A).
70.(B). Bernard-Soulier Syndromeis characterized by moderate, rather than severe,
thrombocytopenia.
71.(B). Aspirin may affect platelets function rather than the count.
72.(D). Severe life-threatening bleeding not responding to all modalities of medical
treatment is an indication
for emergent splenectomy.
73.(D). Splenomegaly is present in only 10% of cases of acute lTP.
74.(E). The most likely diagnosis is factor XIII deficiency.
75.(A). lliopsoas bleeding is life threatening that needs immediate factor replacement
therapy.
76.(B). When mild to moderate bleeding occurs, level of factor Vlll or lX must be raised
to a hemostatic level, in the range of 35-50%.
77.(B). In TAR, thrombocytopenia frequently remits but some cases may require
allogenic bone marrow transplantation.
78.(C). Glanzmanns thrombasthenia is characterized by an abnormal platelets function
and normal platelets count.
79.(E). Spontaneous recovery occurs in the majority of children with ITP;treatment is
required when the platelets count is less than 20,000/ ml.
80.(A).
367
ancer and Benign Tumors
QUESTIONS
SAMA AUUMAILY
2. A 9-year-old boy develops acute myelogenous leukemia (AML) one year after
completion of therapy for soft tissue sarcoma at his right thigh.
Which of the following chemotherapeutic agents is MOST likely the cause of secondary
acute myelogenous leukemia AML in this boy?
A. cyclophosphamide
B. vincristine
C. etoposide
D. doxorubicin
E. cisplatin
3. 0f the following, the genetic syndrome MOST likely associated with increased risk of
optic glioma is
A. Down syndrome
B. neurofibromatosis 1
C. monosomy 7
D. Bloom's syndrome
E. ataxia-telangiectasia
P1905?Wilms tumor
neuroblastoma
medulloblastoma
6. Undescended testis is a risk factor for the development of which of the following
tumors?
A. rhabdomyosarcoma
B. leukemia
C. yolk sac tumor
D. lymphoma
E. neuroblastoma
7. Epstein-Barr virus (EBV) infection is more likely to be associated with all the following
malignancies EXCEPT
A. Burkitt lymphoma
8. nasopharyngeal carcinoma
C. T-cell lymphoma
D. Hodgkin lymphoma
E. hepatocellular carcinoma
9. Hepatitis C virus infection is a risk factor for which of the following malignancy?
A. hepatoblastoma
B. splenic lymphoma
C. Hodgkin lymphoma
D. nasopharyngeal carcinoma
E. cervical carcinoma
10. Nearly all cervical carcinomas contain human papillomavirus (HPV). Which of the
following types is never associated with such malignancy?
A. type6
B. type 16
C. type 18
369
D. type 31
E. type 33
Which
11. chromosomal
ofthefollowing inacute leukemia
lymphoblastic
abnormalities
of childhoodcarrya favorableoutcome?
A. t(12;21)
B. t(4;11)
C. t(9;22)
D. hypodiploidy
E. 11q23
13. Children with ALL who carry poor outcome include all the following EXCEPT
A. age younger than 1 year and older than 10 year
T-cell immunophenotype
hyperdiploidy chromosomal abnormality
thatallow
thefamily
tohave
a
tripis
safe
A. before starting treatment
B. remission induction
C. consolidation
370
D. delayed intensification
E. interim maintenance
16. You are evaluating a 6-year-old child with ALL on interim maintenance phase who
has frequent mucositis and myelosuppression that needs frequent discontinuation of
his treatment.
Of the following, the MOST valuable test for this child is
complete blood count
bone marrow study
a 9-year-old
s-methyltransferase (tpmt) gene
18. Granulocytic sarcoma masses are commonly seen in which type of the following
malignancies?
A. neuroblastoma
B. rhabdomyosarcoma
C. soft tissue sarcoma
D. ALL
E. AML
19. Which of the following types of translocation of childhood AML that typically
associated with granulocytic sarcoma mass?
A. «15:17)
B. t(8:21)
C. inv(16)
D. t(6:9)
E. inv(3)
20. You are explaining the risk of leukemia in children with Down syndrome to medical
students; your discussion will include all the following statements EXCEPT
A. acute leukemia occurs more frequently in children with Down syndrome than
in the general population
371
B. AMLis morecommon
in childrenwith Downsyndromein the 1" 3 yr of life
than ALLascomparedwith the ratio of ALL/AML in general population
C. in childrenwith Down syndromewho have AML, the expected outcome of
treatment is slightlyinferior to that for other children
D. patientswith Downsyndromewho developsacute leukemia demonstrate a
remarkable sensitivityto antimetabolites
E. neonates with Down syndrome may develop a transient leukemia or
myeloproliferativedisorder
21. A healthy 20-day-old male neonate with Down syndrome appears pale;
examination reveals a palpable liver 6 cm below the right costal margin and palpable
spleen 3 cm below the left costalmargin; lab findings include: hemoglobin, 8.8 g/dl;
platelets count, SSOOO/mm3;white blood cell count, 18700/mm3 with 10% blast cells;
bone marrow examination is consistent with acute leukemia.
Of the following,the BESTapproachfor the management is
A intensivechemotherapy
B. low dose chemotherapy
C. pulsesof chemotherapy
D. bone marrowtransplantation
E. closefollow up
in
8 stage IV disease
C. presence of B symptoms
D poor response
to therapymanifested emission
by positron (PET)
tomography
scan positivity
E. presence of bulky mediastinal mass
27. Tumor lysis syndrome (TLS) is a common complication during the treatment of
pediatric malignancies, which of the following malignancies is more likely to manifest
such complication?
A. acute myelogenous leukemia AML
B. nephroblastoma
C. neuroblastoma
D. Burkitt lymphoma
E. Rhabdomyosarcoma
373
29. A 5-year-old child, recently diagnosed with ileocecal Burkitt lymphoma; lab
investigations reveal: serum uric acid 12 mg/dl, serum sodium, 145 meq/dl; serum
potassium, 4.5 meq/dl; serum phosphate 4.4 meq/dl; serum calcium, 8.9 mg/dl; blood
urea, 22 mg/dl; serum creatinine, 0.8 mg/dl.
Of the following, the MOSTeffective treatment is
A. excessive hydration
8. sodium bicarbonate
C. xanthine oxidase inhibitor
0. recombinant urate oxidase
E. no treatment
30. A 6-year-old male child presented with a cerebellar mass; after undergoing
complete surgical resection of the mass, the histology of the mass reveals pilocytic
astrocytoma.
Of the following, the MOSTappropriate next step in the management is
A. radiotherapy
B. chemotherapy
C. observation
0. concomitant chemo-radiotherapy
E. radiotherapy followed by chemotherapy
31. A 6-year-old child complains of bilateral thigh pain, motor weakness and some
sensory deficits of both lower limbs; he has a history of bladder dysfunction over the
past 2 weeks; MRI of the spinal cord shows a mass arising from the fiium terminale and
conus medullaris causing some pressure effect. A CNStumor is suspected.
Which of the following tumor is MOST likely arising in such site?
A. medulloblastoma
B. anaplastic astrocytoma
C. myxopapillary ependymoma
D. choroid plexus carcinoma
E. oligodendroglioma
32. You are discussing the risk of radiotherapy with the parents of a child with
medulloblastoma; the mother has a concern about the late neurological complications
post radiotherapy.
The statement that should be included in the discussion that late neurological sequelae
post radiotherapy is more severe with
A. focal radiotherapy rather than craniospinal irradiation
children with an age of less than 3 year
concomitant chemo-radiotherapy
374
33. The least common late
neurologic sequelae that may be encountered post
craniospinal irradiation for a 9-year-old child with medulloblastoma is
microcephaly
learning disabilities
34. Chemotherapy has a major role in many childhood CNS tumors. In which of the
following tumors chemotherapy is not effective?
A. medulloblastoma
B. pilocytic astrocytoma
C. craniopharyngioma
D. pineoblastoma
E. supratentorial primitive neuroectodermal tumors (SPNETs)
35. Childhood primary brain stem tumors are heterogenous group of tumors; the
outcome usually depends on tumor location.
Which of the following brain stem tumors, depending on the site of tumor, carry the
worst prognosis?
A. focal
B. dorsally exophytic
C. cervicomedullary
D. diffuse intrinsic
E. none ofthe above
36. Extraneural metastasis from primary brain tumors is less likely to occur. Which of
the following primary brain tumor is MOST commonly metastasizing extraneurally?
A. medulloblastoma
B primitive neuroectodermaltumor (PNET)
C. ependymoma
D. malignantglioma
E. choroid plexus carcinoma
375
round
38.Small celltumors
blue roundbluecells
thathave
refertotumors
Theyinclude
histologically. EXCEPT
allthefollowing
A. neuroblastoma
B. non-Hodgkinlymphoma
C. osteosarcoma
D. Ewing sarcoma
E. rhabdomyosarcoma
40. A 1-year-old child develops right eye ptosis, myosis, and loss of sweating; you
suspect neuroblastoma.
0f the following, the MOST valuable investigation to confirm the diagnosis is
A. Computerized tomography (CT) scan of the neck and chest
8. CT scan of the abdomen
C. CT scan of the brain
D. magnetic resonance imaging (MRI) of the brain
E. X-ray the chest
is a highlymalignantchildhoodneoplasm.Metastatic
41. Neuroblastoma
Spreadcan
occurvia local invasionor distant hematogenousor lymphatic routeS.
0f the following,the LEASTcommonsite of metastasesin neuroblastomais
A. long bones
B. bone marrow
C. lung
D. skin
E. liver
canbe associated
42. Neuroblastoma withparaneoplastic Allthe followin
syndrome. g
features
areparaneoplastic ofneuroblastoma
EXCEPT
A. uncontrollablejerking eye and body movements
B. cerebellar ataxia and poor coordination
C. increased sweating and hypertension
D. unilateral ptosis, myosis,and anhidrosis
376
E. profound secretory diarrhea
43. You are evaluating a 6-mo-old girl with a firm abdominal mass. Radiography reveals
a calcified small right suprarenal mass. The tumor is completely resected and
histologically is confirmed as neuroblastoma. Metastatic work-up shows massive liver
involvement, 10% bone marrow involvement by tumor cells, and multiple
subcutaneous nodules involvement. There is no bony involvement. The N-myc
oncogene from the tumor is not amplified.
According to the international neuroblastoma staging system, the infant is stratified as
A. stage I
B. stage II A
C. stage III
D. stage IV
E. stage IV S
44. A 10-mo-old boy has a left suprarenal mass. Surgery is accomplished with complete
surgical removal of the mass as well as the non-adherent ipsilateral lymph nodes;
multiple contralateral lymph nodes biopsies are taken during surgery. The histology
reveals poorly differentiated neuroblastoma with microscopic involvement of the
ipsilateral lymph nodes; the contralateral lymph nodes are negative for tumor cells.
Of the following, the BESTtherapeutic approach for this infant is
A. chemotherapy
B. radiotherapy
C. concomitant chemo-radiotherapy
D. chemotherapy followed by radiotherapy
E. close observation
45. Children with high-risk neuroblastoma have poor survival. Current treatment
consists of all the following EXCEPT
A. surgery
intensive chemotherapy
W90?
radiation
allogenic bone marrow transplantation
differentiating agents (13-cis-retinoic acid)!
46. A 2~year-old male child has an asymptomatic right flank mass discovered
incidentally by the mother while bathing. Suspicion of Wilms tumor is raised.
0f the following, the LEASTlikely investigation to be performed in this child is
A. plain abdominal radiography
B. computerized tomography (CT) scan of the abdomen
C. CT scan of the chest
D. Biopsy of the mass
377
blood
oftheabdominal
imaging vessels
with
Doppler
E. ultrasonography
Wilms
with
forchildren isnot
tumor asItmay
performed
usually upstage
47Biopsy
the disease. _ . fora childwithsuspicion
g renalbiopsy
ofaccomplishln
areindications
Allthefollowing
of Wilms tumor EXCEPT
A. ageof 2-3 year at presentation
andinflammation
ofinfection
B. signs thetumor '
around
abdominal
C. significant nodes
lymph radiologically
enlargement
D. absenceof renalparenchyma radiologically
E. of
appearance
radiological intratumoralcalcrficatron
for a rightflankmass;radiological
childisbeingevaluated
48.A 2-year-old appearance
with rightrenalWilms tumor.Duringradicalnephrectomythere is intra-
is consistent
abdominal tumor rupture stated by the surgeon.
0f the following, the BESTtherapeutic approach for this child is
A. chemotherapy
B. radiotherapy
C. concomitant chemotherapy-radiotherapy
D. another surgery
E observation
49. Although children with Wilms tumor have a favorable prognosis, there are some
adverse factors that affect outcome.
All the following are poor prognosticfactors in children with Wilms tumor EXCEPT
young age at diagnosis
advanced stage
W905?large tumors
anaplastic histology
lossof heterozygosity
at chromosome
1p and 16q
W905?anaplastic histology
relapse to lung only
laterelapse
morethan12mofrom
nephrectomy
378
51. A 2-mo-old male infant has a left flank mass discovered incidentally by the mother;
radiological imaging reveals a left renal mass.
Of the following, the MOST likely diagnosis is
A. nephroblastoma
B. neuroblastoma
C. mesoblastic nephroma
D. clear cell sarcoma
E. rhabdoid tumor of the kidney
52. A healthy 4year-old child has a left flank mass. Computerized tomography (CT)
scan of the abdomen shows a localized left renal mass. Radical nephrectomy is
performed; the histology shows clear cell sarcoma of the kidney.
0f the following, the MOST appropriate next step in the management is
A. CT scan of the chest
8. CT scan of the brain
C. bone scan
D. magnetic resonance imaging (mri) of the brain
E. skeletal survey
53. Renal cell carcinoma (RCC) is rare in children, accounting for <5% of all renal tumors
of childhood.
All the following are true regarding RCCin children EXCEPT
A. patients may present with frank hematuria, flank pain, and/or a palpable mass
B. it can be asymptomatic and detected incidentally
C. it has a propensity to metastasize to the lungs, bone, liver, and brain
D. it can be associated with von hippellindau disease
E. local lymph node involvement has an adverse outcome
54. Alveolar type of rhabdomyosarcoma (RMS) accounts for approximately 1/3 of all
cases of pediatric RMS and carries the poorest prognosis.
Of the following, the MOST common site of involvement by alveolar type RMS is
A. orbit
8. middle ear
C. extremities
D. vagina
E. bladder
379
C. testis
D. orbit
E. paranasalsinuses
bonetumor in childrenand
56- IS "05
the
05te°53m°ma andrequires
t P
' malignant
rimary
common
nt
differe
. .
modalitiesoftreatment
it has
adolescents; subtypes
multiple
includingsurgeryandchemotherapy.
Of the following,the subtypeof osteosarcomaw hich
istreatedbysurgeryaloneis
A. fibroblastic
B. chondroblastic
C. telangiectatic
D. parosteal
E. periosteal
theWORST
57.0f thefollowing, prognostic is
factorin pediatricosteosarcoma
primary pelvic bone tumor
poor histologic response to treatment
62. The serum a-fetoprotein (AFP) level is elevated with some malignant germ cell
tumors (GCTs) especially endodermal sinus tumors; it can be used as a measure of
response to treatment and during follow up after completion of chemotherapy.
However, it is normally elevated during infancy.
At which age does AFP physiologically fall to normal adult level?
three month
eight month
r1909»?
one year
three year
ten year
63. The [3subunit of human chorionic gonadotropin (HCG) is a useful surface marker in
some malignant germ cell tumors (GCTs);it is secreted by syncytiotrophoblasts.
In which of following GCTsthis surface marker is characteristically elevated?
teratoma
W909? germinoma
gonadoblastoma
embryonal carcinoma
64. You are discussing with medical students the role of chemotherapy in malignant
germ cell tumors (GCTs); you state that GCTs are sensitive to some types of
chemotherapy.
Of the following, the MOST effective chemotherapeutic agent in GCTsis
381
vincristine
cyclophosphamide
WP???
cisplatin
methotrexate
cytosineArabinoside
is the mostcommon
66. Hepatoblastoma of the liver;it arisesfrom the
neoplasm
It isof differenthistological
of hepatocytes.
precursors whichhasa direct
classification
correlation with clinical outcome.
Of the following, the histologicaltype that predict the MOST favorable outcome is
A. epithelial type of pure fetal histology
B. epithelial type of pure embryonal histology
C. epithelial type of mixed histology (fetal and embryonal)
D. mixed type of epithelial and mesenchymal elements
E. undifferentiated type
67. Hemangiomas
are the most commonbenigntumors of infancy,
occurringmore
often in the head and neck region.
Ofthefollowing,
theinfantat higherriskof development
of hemangioma
is
A. full term infant
8. male gender infant
c. infantofwomanwhohadchorionic
villussampling
0. infant delivered by cesareansection
E. infant of diabetic mother
382
69. Kasabach-Merritt syndrome is a benign vascular tumor which
may be life-
threatening; it is characterizedby all the following EXCEPT
A. rapidly enlarging lesion
8. thrombocytopenia
C. microangiopathic hemolytic anemia
D. coagulopathy
E. association with infantile hemangiomas
70. A 3-day-old neonate has a large soft, painless mass involving the right lower part of
the head and neck that transilluminate; computerized tomography reveals a cystic
mass infiltrating the neck and intrathoracic region mostly the mediastinum.
0f the following, the BEST modality of treatment for this neonate is
aspiration of mass
surgical resection
71. You are meeting with parents of 12-year-old girl who recently diagnosed with
papillary thyroid carcinoma (PTC). The statement that should be included in your
discussion is
PTC has a grim overall prognosis
chemotherapy is the primary therapy
72. You are examining a 12-year-old female adolescent with a small nevus in the left
thigh; her mother is concerned regarding development of melanoma in future.
All the following findings raise the suspicion of melanoma EXCEPT
A. rapidly enlarging nevus
B. nevus with changing colors
C. nevus with irregular margins
D. easily bleeds nevus
E. Spitz nevus
73. Although melanoma is relatively rare in children, some risk factors may increase its
incidence.
All the following are risk factors for development of melanoma EXCEPT
A. positive family history of melanoma
8. dark skinned child
383
C. giant hairy nevus
nevussyndrome
D. dysplastic
E. xeroderma pigmentosum
carcinoma
74.Nasopharyngeai common
ofthemost
isone tumors
in
nasopharynzeal
pediatric Manyfactors
patients. theprognosis.
mayaffect
theworstoutcome?
carries
Whichofthefollowing
A. elevated (LDH)level
lactatedehydrogenase
B. advanced local disease
C. extensivecervicallymphnodesinvolvement
virus(EBV)DNAlevels
D. Epstein-Barr
E. evidence of distant metastases
mPDF?vertebra
pelvis
femur
76. Langerhans
cellhistiocytosis
(LCH)hasanextremely
variablepresentation.
Singleor
multiple organsmay be affected by the disease.
0f the following,the LEAST
likelyorganaffectedby LCHis
skin
bone
W909?middle ear
lung
lymph nodes
77. In Langerhans
cellhistiocytosis .
. . (LCH)
. SYstemic 5aremore
manifestation 10
occur inpatients
wrthmultisystemdiseasewho
likely
are
I, .
organ patients).
All
the are
risk
following in ( at highriskof
mortality i.e., risk
:sritewe organs
LCH
EXCEPT
liver
reps???
spleen
lung
hematopoietic
system
Cancerand Benign~
Tumours
ANSWERS
SAMA AUUMAILY
385
t(9;22) has dramatically changed by the addition of imatinib to an intensive
chemotherapy backbone.
15.(E).
16.(C). Pharmacogenetic testing of the thiopurine S-methyltransferase (TPMT) gene,
which encodes one of the metabolizing enzymes of mercaptopurine, can identify
patients who are wild type (normal TPMT enzyme activity), heterozygous (slightly
decreased TPMT enzyme activity), or homozygous (low or absent enzyme activity).
Decreased TPMT enzyme activity results in an accumulation of a toxic metabolite of
mercaptopurine and results in severe myelosuppression, requiring dose reductions of
the chemotherapy.
17.(E). Testicular relapse occurs in less than 2% of boys with ALL, usually after
completion of therapy. Such relapse occurs as painless swelling of one or both testes.
The diagnosis is confirmed by biopsy of the affected testis. Treatment includes systemic
chemotherapy and possibly local irradiation. A high proportion of boys with a testicular
relapse can be successfully retreated, and the survival rate of these patients is good.
18.(E).
19.(B). Discrete masses, known as chloromas or granulocytic sarcomas, can occur in the
absence of apparent bone marrow involvement and typically are associated with a
t(8;21) translocation. Chloromas also may be seen in the orbit and epidural space.
20.(C). In AML, patients with Down syndrome have much better outcomes than non-
Down syndrome children, with a >80% long-term survival rate.
21.(E). Approximately 10% of neonates with Down syndrome develop a transient
leukemia or myeloproliferative disorder which usually resolves within the 15! 3 mo of
life. Although these neonates can require temporary transfusion support, they do not
require chemotherapy unless there is evidence of life-threatening complications.
However, those patients require close follow-up, because 20-30% will develop typical
leukemia by 3 yr of life.
22.(E). Twenty-thirty percent of patients who have Down syndrome and who develop
transient leukemia or myeloproliferative disorder will develop typical leukemia (often
acute megakaryocytic leukemia) by 3 yr of life (mean onset, 16 mo).
23.(A). More than 80% of the cases of infantile ALL demonstrate rearrangements of the
MLL gene, found at the site of the 11q23 band translocation, the majority of which are
the t(4;11). This subset of patients largely accounts for the very high relapse rate.
24.(A).
25.(D). Stage II includes involvement of 2 or more lymph node regions on the same side
of the diaphragm (II) or localized involvement of an extralymphatic organ or site and 1
or more lymph node regions on the same side of the diaphragm (llE). HL is subclassified
into A or B categories: A is used to identify asymptomatic patients and B is for patients
who exhibit any 8 symptoms.
26.(D). The primary site of tumor involvement and metastasis pattern varies by
pathologic subtype. Lymphoblastic lymphoma (LBL) commonly manifests as an
intrathoracic or mediastinal supradiaphragmaticmass and also has a predilection for
spreading to the bone marrow and CNS. Burkitt lymphoma (BL) commonly manifests as
abdominal (sporadic type) or head and neck (endemic type) tumor and can metastasize
to the bone marrow or CNS. Diffuse large B-cell lymphoma (DLBCL) commonly
manifests as either an abdominal or mediastinal primary and, rarely, disseminates to
the bone marrow or CNS. ALCL manifests either as a primary cutaneous manifestation
(10%) or as systemic disease (90%) with dissemination to liver, spleen, lung, or
mediastinum. Bone marrow or CNS disease is rare in ALCL.
27.(D). TLS can occur from rapid cell turnover, which is especially common in Burkitt
lymphoma.
28.(B).
29.(D). Recombinant urate oxidase is preferred in patients with a high risk of tumor
lysis. Frequently, only a single dose is needed; however, repeat doses can be given if a
subsequent rise in uric acid is seen.
30.(C). With complete surgical resection the overall survival approaches 80100%.
31.(C). Myxopapillary ependymoma (WHO grade I) is a slow-growing tumor arising
from the filum terminale and conus medullaris; it carries good prognosis.
32.(B).
33.(A). Craniospinal radiation in children younger than 3 yr of age results in severe late
neurologic sequelae, including microcephaly, learning disabilities, cognitive
impairment, and neuroendocrine dysfunction. Similarly, in older children, late
sequelae, such as learning disabilities, neuroendocrine dysfunction, and/or second
malignancies, can occur.
34.(C). There is no role for chemotherapy in craniopharyngioma.
35.(D). Surgical resection is the primary treatment approach for focal and dorsally
exophytic tumors and leads to a favorable outcome. Histologically, these 2 groups
usually are lowgrade gliomas. Cervicomedullary tumors, owing to their location, may
not be amenable to surgical resection but are sensitive to radiation therapy. Diffuse
intrinsic tumors, characterized by the diffuse infiltrating pontine glioma, are associated
with a very poor outcome independent of histologic diagnosis.
36.(A).
37.(D). Ventriculoperitoneal shunts have been known to allow extraneural metastases,
primarily within the peritoneal cavity but also systemically.
38.(C).
39.(B). Hyperdiploidy confers better prognosis if the child is younger than 1 yr of age at
diagnosis. Amplification of MYCN is strongly associated with advanced tumor stage and
poor outcomes. Other chromosomal abnormalities, including loss of heterozygosity of
1p, 11q, and 14q, and gain of 17q, are commonly found in neuroblastoma tumors and
are also associated with worse outcomes.
40.(A). Homer syndrome, characterized by unilateral ptosis, myosis, and anhidrosis, is
associated with a thoracic or cervical primary tumor (e.g., superior cervical ganglion).
Symptoms do not resolve with tumor resection.
387
41. (C). The most common sites of metastasis are the regional or distant lymph nodes,
long bones and skull, bone marrow, liver, and skin. Lung and brain metastases are rare,
occurring in >3% of cases.
42.(D). Horner syndrome results from direct invasion of the neural foramina by tumor,
causing nerve root compression. opsoclonus myoclonusataxia syndrome is of
paraneoplastic nature of autoimmune origin. Neuroblastoma can produce
catecholamines that can cause increased sweating and hypertension; it may release
vasoactive intestinal peptide, causing a profound secretory diarrhea.
43.(E). Infants younger than 1 yr of age also can present in unique fashion, termed
stage 45, with widespread subcutaneous tumor nodules, massive liver involvement,
limited bone marrow disease, and a small primary tumor without bone involvement or
other metastases.
44.(E). The infant has stage 2A and is stratified as low risk. The usual treatment for
children with lowrisk neuroblastoma is surgery for stages 1 and 2 and observation for
stage 45 with cure rates generally >90% without further therapy.
45.(D). Highdose chemotherapy with autologous stem cell rescue, and not allogenic
bone marrow transplantation, is one of the treatment modality for high risk patients.
46.(D). Although biopsy is a reliable diagnostic tool, it is discouraged as it results in
disease upstaging. A core needle biopsy obtained via a posterior approach should be
performed in cases of unusual presentation (older age, signs of infection, inflammation)
or unusual imaging findings (significant adenopathy, no renal parenchyma seen,
intratumoral calcification).
47.(A). Older age, more than 5 years, is an indication to perform renal biopsy.
48.(C). The child is stratified as stage III; so he needs chemo-radiotherapy.
49.(A). Prognostic factors for risk-adapted therapy include age, stage, tumor weight,
and loss of heterozygosity at chromosomes 1p and 16q. Histology plays a major role in
risk stratification of WT. Absence of anaplasia is considered a favorable histologic
finding.
SO.(C). Factors associated with a favorable outcome after relapse include low stage
(l/ll) at diagnosis, treatment with vincristine and actinomycin 0 only, no prior
radiotherapy, favorable histology, relapse to lung only, and interval from nephrectomy
to relapse 12 mo or longer.
51.(C). Mesoblastic nephroma is the most common solid renal tumor identified in the
neonatal period. Most of the patients are diagnosed before 3 mo of age, whereas
Wilms tumor is rarely diagnosed before 6 mo of age. Radical nephrectomy is the
treatment of choice and may be sufficient by itself.
52.(C). Bone is the most common site of distant metastasis in clear cell sarcoma of the
kidney followed by lung, abdomen, retroperitoneum, brain, and liver.
53.(E). Unlike the case for adult RCC, local lymph node involvement is not a poor
prognostic indicator in pediatric RCC.Nephrectomy alone may be adequate for early/
stage RCC.
54.(C). Alveolar tumors occur most often in the trunk and extremities.
388
55.|E). Favorable sites include female genital, paratesticular, and head and neck
(nonparameningeal) regions; all other sites are considered unfavorable. Involvement of
paranasal sinuses by rhabdomyosarcoma is considered as unfavorable site.
56.(Dl. Parosteal osteosarcoma is a low~grade, well-differentiated tumor that does not
invade the medullary cavity and most commonly is found in the posterior aspect of the
distal femur. Surgical resection alone often is curative in this lesion, which has a low
propensity for metastatic spread.
57.(C). All the mentioned factors carry poor prognosis, but multiple bony metastases at
the time of diagnosis is the worst.
58.(D). Osteosarcoma is a radio-resistant tumor.
59.(C). Osteoid osteomas are diagnosed between 5 and 20 yr of age. The clinical
pattern is characteristic, consisting of unremitting and gradually increasing pain that
often is worst at night and is relieved by aspirin. Boys are more affected than girls.
Radiographs are distinctive, showing a round or oval metaphyseal or diaphyseal
lucency (nidus) surrounded by sclerotic bone.
60.(B). Imaging studies are not diagnostic, and biopsies are contraindicated. Indirect
ophthalmoscopy with slit-lamp evaluation can detect retinoblastoma tumors, but a
complete evaluation requires an examination under general anesthesia by an
experienced ophthalmologist to obtain complete visualization of both eyes, which also
facilitates photographing and mapping of the tumors.
61.(B). Retinoblastoma can be either hereditary or sporadic. Hereditary cases usually
are diagnosed at a younger age and are multifocal and bilateral, whereas sporadic
cases are usually diagnosed in older children who tend to have unilateral, unifocal
involvement. All lst-degree relatives of children with known or suspected hereditary
retinoblastoma should have retinal examinations to identify retinomas or retinal scars.
62.(B). Infants normally have higher levels of AFP, which fall to normal adult levels by
about age 8 mo; consequently, high AFP levels must be interpreted with caution in this
age group.
67.(C). The risk of hemangioma is 3-5 times higher in girls than boys. The risk is doubled
in premature infants and 10 times higher in offspring of women who had chorionic
villus sampling.
68.(D). An ultrasonographicscan or MRI of the liver should be performed if multiple
cutaneous lesions are present.
389
69.(E). All the features of Kasabach-Merritt syndrome results from platelet and red
blood cell trapping and activation of the clotting system within the vasculature of the
hemangioma. This syndrome is associated with kaposiform hemangioendotheliomas or
tufted angiomas but not with infantile hemangiomas.
70.(C). Localized cystic hygroma lesions may be surgically resected, but this can be
difficult, owing to their infiltrative nature. Recurrence is common with incompletely
resected lesions. Aspiration can provide temporary relief in an emergency and
reaccumulation will occur. Treatment by injection of sclerosing agents (by streptococcal
immunotherapeutic agent OK-432) is the treatment of choice; its use will prevent the
need for surgery in most cases. Laser therapy is not used in such age. Although
spontaneous regression has been reported but is not typical.
71.(D). PTC has an excellent overall prognosis even in the presence of metastatic
disease. The primary therapy is a total thyroidectomy and lymph node dissection. 131I
is used postoperatively to treat distant metastasis and unresectable residual neck
disease. The TSH level is suppressed by giving supraphysiologic levothyroxine as TSH
stimulates tumor growth. Long-term follow-up involves monitoring of thyroglobulin
tumor markers.
72.(E). Spitz nevus is a benign harmless skin lesion. However, making the distinction
from diagnosis of melanoma can be difficult.
71(8). Patients with fair skin are at particularly high risk for development of
melanoma.
74.(E). Most pediatric patients present with advanced locoregional disease. LDH is
elevated but it is not specific. Epstein-Barr virus DNA levels correlate with disease
stage, have prognostic valueand can be used to monitor for recurrence.The outcome
depends on the extent of disease; patients with distant metastases have a very poor
prognosis.
7S.(A). LCH has an extremely variable presentation. The skeleton is involved in 80% of
patients and may be the only affected site, especially in children older than 5 yr of age.
Bone lesions may be single or multiple and are seen most commonly in the skull.
76.(D). Bone is the most common site of involvement by LCH. About 50% of patients
experience skin involvement at some time during the course of disease. Localized or
disseminated lymphadenopathy is present in approximately 33% of patients. Otitis
media is present in 30-40% of patients. In 10-15% of patients, pulmonary infiltrates are
found on radiography. The lesions may range from diffuse fibrosis and disseminated
nodular infiltrates to diffuse cystic changes.
77.(A).
390
ep ro ogv 1
QUESTIONS
91,009?
bowmans capsule
parietal epithelial cell
visceral epithelial
PROPS?
creatinine
albumin
glucose
4. In contrast to the concentration of blood urea nitrogen, the serum creatinine level is
primarily influenced by
A. state of hydration
B nitrogen balance
C. muscle mass
D. hemorrhage
E. vomiting
391
glomerulonephritis
C. mesangioproliferative
D. diabeticnephropathy
E. classII lupus nephritis
cells
per microliterof
'5damed
' ' 35 the P
6.Hematuna beseenin urinarydipstick
of
resence at le 5
ast redblood
inallthe
reading
.
followmg
resultsmay
urine,false-positive
EXCEPT
an alkaline urine (pH > 8)
contaminationwith hydrogenperoxide
isalways
7.Rhabdomyolysis asit mayleadto acuterenalinjury,It
significant
clinically
can occurs secondary to
A. severe electrolyte abnormalities
8. bacterial endocarditis
C. transfusion reactions
D. mycoplasma infection
E. autoimmune hemolytic anemia
91.005?
gross hematuria that is bright red or pink
the presence of leukocytes cast
normal urinary RBC morphology
wpnw?proteinuria
hypertension
persistent high serum complement C3
15. Thin basement membrane disease TBMD is defined by the presence of persistent
microscopic hematuria and isolated thinning of the GBM on electron microscopy, all
the following are true EXCEPT
A. significant proteinuria is rare
B. episodic gross hematuria can be present
C. may be transmitted as an autosomal dominant trait
D. heterozygous mutations in the COL4A4 genes
393
E. renal insufficiency is common
best cutaneous
testtodocument is
infection
streptococcal
16.The single
A. low serum C3 level
B. elevated antistreptolysin O titer
C. positive streptozyme screen
D. antideoxyribonuclease8 level
E. depressed serum CH50
19. Acute post- infectious glomerulonephritis is one of the most common glomerular
causes of gross hematuria in children.
Of the following, the TRUE statement is
A. can follow fungal infection
8. early systemic antibiotic, eliminate the risk of glomerulonephritis
C. all family members of patient need antibiotic treatment
D. antibiotic affect the natural history of the disease
E. recurrences are common
-
20. The MOST common etiology of seconda 'y membra "05 "Ephropathv
(MN)m
children
is
A neuroblastoma
B. measles
C. systemic lupus erythematosis
D. chronic hepatitis C
394
E. penicillin
glomerulonephritis is
A. lesscommonthantype II membranoproliferative
glomerulonephritis
8. not to be mediated by immune complexes
C. associated with partial lipodystrophy
D. called dense deposit disease
E. characterized by low C3 complement level
91.0.05?
24. WHO
60%of patients
40% of patients
20% of patients
395
A. isolated microscopichematuria
B. insignificant proteinuria
C. severe systemic manifestations
0. >50% crescents on renal biopsy
E. lgA deposits intensity
30. In hemolytic-uremic
syndrome,thromboticmlcroangiopathles
are associatedwith
all the following EXCEPT
A. genetically determined factor H deficiency
B. neuraminidase-producing Streptococcus pneumoniainfection
C. systemic lupus erythematosls
D. malignancy
E. hypotension
396
stool cultureis often positive in patients who have diarrhea-associated HUS
WP?!
kidney biopsy is rarely indicated to diagnose HUS
partial thromboplastin and prothrombin times are usually low
Coombs test is usually negative in in pneumococciinduced HUS
34. Renal cortical necrosis is a rare cause of acute renal failure occurring secondary to
extensive ischemic damage of the renal cortex.
Of the following, the lEAST common cause is
A. perinatal asphyxia
severe congenital heart disease
W90?
severe hemolytic-uremic syndrome
amniotic fluid embolism
infectious endocarditis
35. All the following causes hematuria associated with rapid development of
microangiopathic hemolytic anemia or enlargement of the kidney(s) EXCEPT
A. nephroticsyndrome
B. renal veinthrombosis
C. polycystic kidney disease
D. Wilmstumor
E. hemolytic~uremic syndrome
397
E. Addisons disease
manifestations
37.Clinical SSNincludeall the fOIIOWing
of sicklecellnephfopathy
EXCEPT
A. hematuria
B. polyuria
C. hemolytic-uremic syndrome
D. renal tubular acidosis
E. nephrotic-range proteinuria
38. Autosomal dominant polycystic kidney disease is the most common hereditary
human kidney disease, all the following are true EXCEPT
A. gross or microscopic hematuria
B. may be seen in neonates
C. abnormal renal sonography in the absence of symptoms
D. is a multiorgan disorder
E. right sided valvular heart disease
39. Hemorrhagic cystitis can occur in response to all the following EXCEPT
cyclophosphamide
adenovirus infection
WPPF?cyclosporine
polyoma BK virus infection
amyloidosis
40. All the following are treatment options of hemorrhagic cystitis EXCEPT
A. antibiotic
B. intensive intravenous hydration
C. forced dieresis
D. analgesia
E. spasmolytic drugs
398
C. contamination of the urine with blood
D. highly concentrated urine specimen
E. contamination of the urine with antiseptic
43. Which of the following methods offer more precise information regarding urine
protein excretion?
frothy urine
dipstick testing
399
48.Nephrotic state
isahypercoagulable
syndrome from
resulting
A. hemodilution
plateletnumber
B. decreased
incoagulation
C. changes levels
factor
inhepatic
D. decrease productionoffibrinogen
III
E. highlevelof antithrombin
49.When ofminimal
forfirstattack
youinvestigate change syndrome
nephrotic
(MCNS),oneof the followingisTRUE
hematuria
A. microscopic ispresentin20%ofchildren
: creatinine
B. spoturineprotein be<2.0
ratioshould
C. serumcomplementlevelsare low
D. renalbiopsyisroutinelyperformed
E. 2+ proteinuria
52.Toreduce
theriskofserious
infections
inchildren
withnephrotic ' allthe
syndrome
immunizations
following canbeusedEXCEPT
A. pneumococcal 13-valentconjugantvaccine
B. influenzavaccineannuallyto the child
C. pneumococcal
23-valentpolysaccharide
vaccine
D. varicella-zostervaccine
E. influenza
vaccination totheirhousehold
annually contacts
53.Afterachievement
ofremission
inchildren
withnephroticsyndrome,
A. restrict their activities they should
8. restrict sodium intake
400
C. be considered chronically ill
D. maintain an unrestricted diet
E. decrease water/fluid intake
401
enzymeinhibitors
E. angiotensin-converting
59.Urinalysis withFanconi
inpatients syndrome allthefollowing
mayshow EXCEPT
A. urine pH isalkaline
B. glycosuria
C. uricosuria
D. phosphaturia
E. elevated urinary sodium
63. Urinary indices in patients with type lV RTA include all the following EXCEPT
A. may be alkaline or acidic
B. elevated sodium level
C. inappropriately low potassium level
D. foul-smell
E. aminoaciduria
402
B. can be caused by hypokalemia
C. mutations in the AQPZ gene in X-linked form
D. and crying are uncommon features
irritability
E. secondary form present with hyponatremia
65. All the following are options in the treatment of nephrogenicdiabetes insipidus
EXCEPT
free access to water
furosemide
meow? hydrochlorothiazide
amiloride
indomethacin
68. All the following are options in the treatment of Bartter syndrome EXCEPT
A. potassium supplementation
B. aldosterone antagonist
C. high-sodium diet
D. lndomethacin
E. kidney transplantation
403
Esecretion
E. elevatedprostaglandin
70.Thejuvenile ofinherited
JNisa group
nephronophthisis determined
genetically
renal
cystic diseases, EXCEPT
withallthefollowing
itcanbepresented
oliguria
unexplained anemia
91.009?
growth failure
chronic renal failure
retinal degeneration
72. Medical management of acute kidney injury include all the following EXCEPT
A. bladder catheter should be placed immediately
8. intravenous administration of boluses isotonic saline
C. furosemide may be administered as a single lV dose
D. fluid restriction is essential if there is no response to a diuretic challenge
E. mannitol is not effective in prevention of pigment induced renal failure
73. Hyperkaiemia can lead to cardiac arrhythmia, cardiac arrest, and death. Serum
potassium can be lowered by all the following EXCEPT
A. regular insulin with glucose
8. calcium gluconate
C. sodium bicarbonate
D. Kayexalate
E. Dialysis
74.Treatment
of hypocalcemia
inacutekidneyinjuryinclude
allthefollowingEXCEPT
A. low-phosphorus diet
3. intra venous phosphate binders
C. calciumshouldnot be given
intravenously
D. sevelamer
E. avoid aluminum-based binders
404
75. Indications for dialysis in acute kidney injury include all the following EXCEPT
A. anuria/oliguria
B. persistent hypercalcemia
C. severe metabolic acidosis
D. volume overload
E. blood urea nitrogen >100-150 mg/dl
76. Many patients with acute kidney injury require dialysis support for1-3 wk, which of
the following is TRUE statement
A. Intermittent hemodialysis is useful in patients with unstable hemodynamic
status
B. continuous renal replacement therapy is useful in patients with stable
hemodynamic status
C. in peritoneal dialysis ,hypo-osmolar dialysate is infused into the peritoneal
cavity
D. anticoagulation is not necessary in peritoneal dialysis
i urea and creatinine clearance more with peritoneal dialysis than intermittent
hemodialysis
78. Pathogenesis of chronic kidney disease (CKD) include all the following EXCEPT
hyperfiltration injury
hyperkalemia
POP? proteinuria
hypertension
hyperphosphatemia
79. End~stage renal disease (ESRD) represents the state in which a patients renal
dysfunction has progressed to the point at which homeostasis and survival can no
longer be sustained with native kidney function and maximal medical management.
Of the following, the TRUE statement is
A. the ultimate goal for children is dialysis
B. renal replacement therapy be initiated at stage 5 CKD
C. the time to actually initiate dialysis include impaired school performance
D. peritoneal dialysis is performed 3 times weekly
5- peritoneal dialysis use cycler-driven therapy increases the risk of peritonitis
405
therapy
istheoPtimal With
forchildren end-stage
renal
transplantation
80Kidney
disease, trueare
allthefollowing .
. metastatic
EXCEPI: ~
ispreexrsting
contraindication
A relative malignancy
B.successful toimprovement
leads
transplantation in growth
linear
their
C. preemptive mean wuthout
transplantation
transplantation dialy5|s
prior
result
outcomes
D. optimal >15
whenthechildweighs kg
beforesurgery
shouldbeaddressed
E. urologicproblems
83. Before renal transplantation, hemoglobin levels should be maintain at the level
between
A. 7 and 8 g/dL
B. 9and 10 g/dL
C. 11 and 12 g/dL
D. 13 and 14 g/dL
E. 15 and 16 g/dL
Infectionsneedto be identifiedandtreatedbeforerenal
8;. transplantation.Infectious
disease screeningincludesall the following EXCEPT
A. tuberculosis skin test
B. cytomegaloviruslgG
C. measles antibody
406
D. hepatitis A serology
E. varicella titer
87. 0f the following, the BEST choice for maintenance immunosuppresslon in renal
transplantation is
A. cyclosporin
B. basiliximab
C. daclizumab
D. rituximab
E. belatacept
88. Th e side-effect profile of cyclosporine in children include all the following EXCEPT
A. hypertrichosis
8. gingival hyperplasia
C. neutropenia
D. coarsening facial features
E. hyperlipidemia
407
A. sodiumrequirement:0.6 xweightIn Its X(125- serum sodiumin mEq/L)
8. blood anion gap=[Na] - [Cl +HC03]
C. blood anion gap=INa - Cl -HC03]
D. urine anion gap ([unne Na + urine K+] ~ urine CH
6. estimated GFR = 0.43 x height in m/serum creatlnine in mg/dl.
ep ro ogy
ANSWERS
H'i BAH-
1.(B).
2.(B).
3.(D). The ultrafiltrate, which is cell free, contains all of the substances in plasma
(electrolytes, glucose, phosphate, urea, creatinine, peptides, and low-molecular-weight
proteins except proteins having a molecular weight of 268 kDa [such as albumin and
globulinsl).
4.(C). Creatinine is affected by sex, height, muscle mass, bilirubin, and red blood cell
hemolysis, while the concentration of blood urea nitrogen is affected by state of
hydration and nitrogen balance.
5.(B). Membranous nephropathy result from epithelial cell injury, epithelial cells, in
conjunction with basement membrane, allow filtration of plasma solutes but retard
passage of cells and plasma proteins. Disease related to these cells is typified by the
presence of sub epithelial deposits and flattening of the foot processes that engage the
basement membrane, resulting in disruption of the filtration barrier and proteinuria.
6.(C). False negative results can occur in the presence of formalin (used as a urine
preservative) or high urinary concentrations of ascorbic acid (i.e., in patients with
vitamin C intake >2000 mg/day).
7.(A). Rhabdomyolysis can occurs secondary to viral myositis, crush injury, severe
electrolyte abnormalities (hypernatremia, hypophosphatemia), hypotension,
disseminated intravascular coagulation, toxins (drugs, venom), metabolic disorders of
muscles, and prolonged seizures. While other distractors are causesof hemolysis and
hemoglobinuria.
8.(A). Hematuria from within the glomerulus is often associated with urinary
microscopic findings of RBC casts, and deformed urinary RBCs (particularly
acanthocytes), brown, cola- or tea-colored, or burgundy urine, and proteinuria >100
mg/dLvia dipstick. Hematuria originating within the tubular system may be associated
with the presence of leukocytes or renal tubular casts. Lower urinary tract sources of
hematuria may be associated with gross hematuria that is bright red or pink, terminal
hematuria (gross hematuria occurring at the end of the urine stream), blood clots,
normal urinary RBC morphology, and minimal proteinuria on dipstick (<100 mg/dL).
9.(E). Renal biopsy is indicated for children with persistent low serum complement C3.
10.(D). Gross hematuria often occurs within 1-2 days of onset of an upper respiratory
or gastrointestinal infection, in contrast to the longer latency period observed in acute
post infectious glomerulonephritis, and may be associated with loin pain. Proteinuria is
often <1000 mg/24 hr in patients with asymptomatic microscopic hematuria. Mild to
moderate hypertension is most often seen in patients with nephritic or nephrotic
syndrome, but is rarely severe enough to result in hypertensive emergencies. Normal
serum levels of C3 in lgA nephropathy help to distinguish this disorder from post
streptococcal glomerulonephritis. Serum lgA levels have no diagnostic value because
they are elevated in only 15% of pediatric patients.
11.(E). Recurrent macroscopic hematuria is a good prognostic indicator.
12.(D). Angiotensin-converting enzyme inhibitors and angiotensin II receptor
antagonists are effective in reducing proteinuria and retarding the rate of disease
progression when used individually or in combination. Corticosteroids reduce
proteinuria and improve renal function in those patients with a glomerular filtration
rate>60 mL/min/m2.To date, additional immunosuppression with cyclophosphamide or
azathioprine has not appeared to be effective, but further randomized clinical trials are
in progress. Tonsillectomy has been used as treatment for lgA nephropathy in many
countries including Japan. Performing a tonsillectomy in the absence of significant
tonsillitis in association with lgA nephropathy is currently not recommended until
appropriate prospective, controlled trials have been performed and demonstrate
efficacy. Patients with lgA nephropathy may undergo successful kidney transplantation.
Although recurrent disease is frequent, allograft loss caused by lgA nephropathy occurs
in only 15-30% of patients.
13.(A). All patients with AS have asymptomatic microscopic hematuria, which may be
intermittent in girls and younger boys. Progressive proteinuria, often exceeding 1 g/24
hr., is common by the 2nd decade of life and can be severe enough to cause nephrotic
syndrome. Bilateral sensor neural hearing loss, which is never congenital, Ocular
abnormalities, which occur in 3040% of patients with X-linked AS, include anterior
lentic onus (extrusion of the central portion of the lens into the anterior chamber),
macular flecks, and corneal erosions. Leiomyomatosis of the esophagus,
tracheobronchial tree, and female genitals in association with platelet abnormalities
has been reported, but is rare.
14.(B). Absence of epidermal basement membrane staining for the (15 chain of type IV
collagen in male hemi zygotes and discontinuous epidermal basement membrane
staining in female heterozygotes on skin biopsy is pathognomonic for X~|inked AS and
can preclude diagnostic renal biopsy.
15.(E). Rare cases of TBMD progress, and such patients develop significant proteinuria,
hypertension, or renal insufficiency.[ heterozygous mutations in the COL4A3 and
COL4Agenes, which encode the (13 and a4 chains of type IV collagen present in the
GBM, result in TBMD while homozygous mutations in these same genes result in
Autosomal recessive Alport syndrome].
16.(D). Four differences in APSGN follow throat or skin infections:
Post streptococcal GN commonly follows streptococcal pharyngitis during cold-weather
months and streptococcal skin infections or pyoderma during warm-weather months.
Epidemics of nephritis have been described in association with throat (serotypes M1,
M4, M25, and some strains of M12) and skin (serotype M49) infections.
410
The typical patient develops an acute nephritic syndrome 1-2 wk. after an antecedent
streptococcal pharyngitis or 36 wk. after a streptococcal pyoderma.
The antistreptolysin 0 titer is commonly elevated after a pharyngeal infection but
rarely increases after streptococcal skin infections.
17.(E). Acute nephritic syndrome 90%, nephrotic syndrome 10-20%, acute renal failure
50%, the antistreptolysin O titer is commonly elevated after a pharyngeal infection but
rarely increases after streptococcal skin infections, hypertension 70%.
18.(D). Renal biopsy is considered when hematuria and proteinuria, diminished renal
function, and/or a low C3 level persist more than 2 mo. after onset.
19.(A). Acute GN can occur after certain fungal, rickettsial, protozoan, parasitic, or viral
diseases (influenza and parvovirus) thatis why the terms APSGN and acute post
infectious GN are used synonymously. Early systemic antibiotic therapy for
streptococcal throat and skin infections does not eliminate the risk of GN. Family
members of patients with acute GN, especially young children, should be considered at
risk and be cultured for group A B-hemolytic streptococci and treated if positive. Family
pets, particularly dogs, have also been reported as carriers. A 10 day course of systemic
antibiotic therapy with penicillin is recommended to limit the spread of the
nephritogenic organisms, antibiotic therapy does not affect the natural history of
APSGN, and recurrences are extremely rare.
20.(C). MN also associated with chronic hepatitis B infection
and congenital syphilis,
other chronic infections, including malaria, which is likely the most common cause of
nephrotic syndrome worldwide. Certainmedications, such as penicillamine and gold, or
chronic factor replacement in patients with hemophilia. Rare causes such as
neuroblastoma, or other idiopathic systemic diseases.
21.(A). A subset of patients with MN present with a major venous thrombosis,
commonly renal vein thrombosis, this well-known complication. Approximately 20% of
children have hypertension at presentation, most patients also have microscopic
hematuria and only rarely present with gross hematuria. MN is most common in the
2nd decade of life, but it can occur at any age, including infancy.The disease usually
manifests as nephrotic syndrome and accounts for 2-6% of all cases of childhood
nephrotic syndrome.
22.(E). Type II MPGN (dense deposit disease) is less common than type I
membranoproliferative glomerulonephritis, not to be mediated by immune complexes,
demonstrate an associated systemic disease called partial lipodystrophy, serum C3
complement levels are low in the majority of cases(while in membranous nephropathy
is normal), Patients present in equal proportions with nephrotic syndrome, acute
nephritic syndrome, or persistent asymptomatic microscopic hematuria and
proteinuria (while in membranous nephropathy usually manifest as nephrotic
syndrome).
23.(B). Renal disease in childhood SLE is present in up to 80% of patients while
approximately 50% of patients with HSP develop renal manifestations
411
24.(D). Renal biopSy should be performed in all patients with SLE because
histopathologic findings are used to determine the selection of specific
immunosuppressive therapies.
25.(E). Plasmapheresis is ineffective in lupus nephritis unless there is accompanying
thrombotic thrombocytopenic purpura or antineutrophilic cytoplasmic antibody
associated disease.
26.(D). Spontaneous and complete resolution of the nephritis typically occurs in the
majority of patients with mild initial manifestations (isolated hematuria with
insignificant proteinuria), and the severity of the systemic manifestations does not
correlate with the severity of the nephritis.
27.(D). Plasmapheresis may also benefit patients with ANCAassociated crescentic GN,
in particular those with the most severe renal dysfunction at presentation. The possible
benefits of plasmapheresis in other forms of RPGN are unclear.
28.(E).
29.(E).
30.(E). Malignant hypertension.
31.(C). Kidney biopsies are only rarely performed in HUS because the diagnosis is
usually established by clinical criteria and the risks of biopsy are significant during the
active phase of the disease. The organisms that cause HUS may be rapidly cleared,
therefore, the stool culture is often negative in patients who have diarrheaassociated
HUS, Partial thromboplastin and prothrombin times are usually normal, the Coombs
test is negative, with the exception of pneumococci-induced HUS, where the Coombs
test is usually positive.
32.(D). Eculizumab is an anti-C5 antibody that inhibits complement activation, a
pathway that contributes to active disease in some forms of atypical familial HUS,
Eculizumab is FDA approved for the treatment of atypical HUS, while initial reports
suggested that eculizumab provided benefit in patients with diarrhea associated HUS,
subsequent systematic analysis showed no benefit from either plasma exchange or
eculizumab.
33.(A). Nephrotic syndrome-- penicillamine while interstitial nephritis--- penicillin
34.(E). Less-common causes of cortical necrosis include malaria, extensive burns,
snakebites, infectious endocarditis, and medications (e.g., nonsteroidal anti-
inflammatory agents). Acute renal cortical necrosis has also been reported to occur in
systemic lupus erythematosisassociated antiphospholipid antibody syndrome.
35.(A). These include hemolytic uremic syndrome, hydronephrosis, polycystic kidney
disease, Wilms tumor, and intrarenall abscess or hematoma. All patients should be
evaluated for congenital and acquired hypercoagulable states.
3648). Hypercalciuria can accompany conditions resulting in hypercalcemia, such as
hyperparathyroidism, vitamin D intoxication, immobilization, and sarcoidosis.
Hypercalciuria may be associated with Cushing syndrome, corticosteroid therapy,
tubular dysfunction secondary to Fanconi syndrome (Wilson disease,
oculocerebrorenalsyndrome), Williams syndrome, distal renal tubular acidosis, or
412
Bartter syndrome. Oral thiazide diuretics can normalize urinary calcium excretion by
stimulating calcium reabsorption in the proximal and distal tubules.
37.(C). Polyuria caused by a urinary concentrating defect, renal tubular acidosis, and
proteinuria associated with the glomerular lesions. Approximately 20-30% of patients
with sickle cell disease develop proteinuria, Nephrotic-range proteinuria with or
without clinically apparent nephrotic syndrome occurs in up to 30% of patients with
SSN, and when present generally heralds progressive renal failure.
38.(E). Mitral valve prolapse is seen in approximately 12% of children; aortic and
coronary artery aneurysms and aortic valve insufficiency are noted in affected adults. It
is a systemic disorder with possible cyst formation in multiple organs (liver, pancreas,
Spleen, brain) and the development of saccular cerebral aneurysms.
39.(C). The hemorrhagic cystitis can occur in response to chemical toxins
(cyclophosphamide, penicillins, busulfan, thiotepa, dyes, insecticides), viruses
(adenovirus types 11 and 21 and influenza A), radiation, and amyloidosis. The polyoma
BK virus, present latently in immunocompetent hosts, is associated with the
development of drug-induced cystitis in immunosuppressed patients.
40.(A).
41.(A). Findings on urine culture, intravenous pyelography, voiding cystourethrography,
and cystoscopy are normal in most patients, hematuria generally resolves within 48 hr.
after cessation of exercise, exercise hematuria is less common in females and can be
associated with dysuria.
42.(B). Dilute urine or a large volume of urine output or in disease states in which the
predominant urinary protein is not albumin cause falsenegative test results.
4345).
44.(C). Defined contributing factors include a temperature >38.3°C (101°F), exercise,
dehydration, cold exposure, heart failure, seizures, or stress.
45.(C). The absence of proteinuria (dipstick negative or trace for protein; and a normal
ratio of urinary protein [mg/dL] to urinary creatinine [mg/dL]= [uPr/uCr] <0.2) on the
first morning urine sample for 3 consecutive days confirms the diagnosis of orthostatic
proteinuria, children with this condition are usually asymptomatic; hematuria,
hypertension, hypoalbuminemia, edema, and renal dysfunction are absent, the cause
of orthostatic proteinuria is unknown, although altered renal hemodynamics and
partial left renal vein obstruction in the upright, lordotic position have been proposed
as possible causes.
46.(A). Infections causes secondary nephrotic syndrome is : Endocarditis, Hepatitis B,
C,HlV-1, infectious mononucleosis, malaria, syphilis (congenital and secondary),
toxoplasmosis, schistosomiasis, filariasis.
47.(E).
48.(C). Vascular stasis from hemoconcentration,intravascular volume depletion ,
increased platelet number and aggregability, increase in hepatic production of
fibrinogen,and low level of antithrombin III, cause hypercoagulable state.
413
49.(A). A spot urine protein : creatinine ratio should be >2.0, serum complement levels
are normal, a renal biopsy is not routinely performed if the patient fits the standard
clinical picture of MCNS, and the urinalysis reveals 3+ or 4+ proteinuria.
SO.(E). Symptomatic volume overload, hypertension, heart failure, and pulmonary
edema, is a potential complication of parenteral albumin therapy, particularly when
administered as rapid infusions.
51.(C). Steroid-resistant nephrotic syndrome is usually caused by focal segmental
glomerulosclerosis (80%), minimal change nephrotic syndrome, or
membranoproliferativeglomerulonephritis.
52.(D). Following close contact with varicella infection, give immune compromised
children on immunosuppressive agents varicella-zoster immune globulin if available.
S3.(D). To minimize the psychologic effects of the condition and its therapy, children
with idiopathic nephrotic syndrome should not be considered chronically ill and should
participate in all ageappropriate childhood activities.
54.(B). Congenital nephrotic syndrome may be classified as primary or as secondary to
a number of etiologies such as in utero infections (cytomegalovirus, toxoplasmosis,
syphilis, hepatitis B and C, HIV).
55.(E). Renal transplantation is the definitive treatment of congenital nephrotic
syndrome, though recurrence of the nephrotic syndrome has been reported to occur
after transplantation.
56.(A). Hyperkalemia RTA---- Trimethoprim/sulfamethoxazole.
57.(D). Ocular presentations include photophobia, retinopathy, and impaired visual
acuity. Patients also can develop hepatosplenomegaly, and delayed sexual maturation.
S8.(D). Cysteamine, which binds to cystine and converts it to cysteine. Oral cysteamine
does not achieve adequate levels in ocular tissues, so additional therapy with
cysteamine eye drops is required. Patients with growth failure that does not improve
with cysteamine might benefit from treatment with growth hormone. Kidney
transplantation is a viable option.
59. (A).The urine pH is acidic (<5.5) because distal acidification mechanisms are intact
in these patients.
SO.(E).Hypercalciuria, hypocitraturia, and sensorineural deafness are seen in distal RTA
while phosphate and massive bicarbonate wasting are characteristic of proximal RTA.
61.(B). Medullary sponge kidney is characterized by cystic dilation of the terminal
portions of the collecting ducts as they enter the renal pyramids, Ultrasonographically,
patients often have medullary nephrocalcinosis, complications include nephrolithiasis,
pyelonephritis, hyposthenuria (inability to concentrate urine), and distal RTA.
61(8). Type IV RTA occurs as the result of impaired aldosterone production
(hypoaldosteronism) or impaired renal responsiveness to aldosterone
(pseudohypoaldosteronism), proximal RTA pathogenesis is massive bicarbonate
wasting. Acidosis results because aldosterone has a direct effect on the H+/ATPase
responsible for hydrogen secretion. in addition, aldosterone is a potent stimulant for
potassium secretion in the collecting tubule; consequently, lack of aldosterone resulté
414
in hyperkalemia. This further affects acidbase status by inhibiting ammoniagenesis
and, thus, H+ excretion.
63.(E). Urinary indices in patients with Fanconi syndrome demonstrate varying degrees
of aminoaciduria, and elevated urinary sodium or potassium; while in type IV RTA
elevated urinary sodium levels with inappropriately low urinary potassium levels reflect
the absence of aldosterone effect.
6MB). The most common pattern of inheritance in congenital NDI is an Xlinked
recessive disorder, mutations in the AQPZ gene have been identified in patients with
the rarer autosomal dominant and recessive forms, lrritability and crying are common
features, patients with the secondary form generally present later in life, primarily with
hypernatremia and polyuria.
65.(B).
66.(E). Blood pressure is usually normal. Patients with the antenatal form can have
severe salt wasting, resulting in dehydration and hypotension.
67.(B). Urinary calcium levels are typically elevated, as are urinary potassium and
sodium levels, nephrocalcinosis resulting from Hypercalciuria may be seen on
ultrasound examination. Serum renin, aldosterone, and prostaglandin E levels are often
markedly elevated; hypomagnesaemia is seen in a minority of patients but is more
common in Gitelman syndrome. Renal biopsy is rarely performed to diagnose this
condition.
68.(E).
69.(B). The urinary calcium level is usually very low (in contrast to the elevated urinary
calcium level often seen in Bartter syndrome), hypokalemia, aldosterone levels usually
normal, prostaglandin E secretion is not elevated.
70.(A). Patients with JN typically present with polyuria, anemia that is seemingly
disproportionate to the degree of renal insufficiency, JN often have characteristic small
cysts in the corticomedullary region.
71.(D). Patients whose urine shows an elevated specific gravity (>1.020), elevated urine
osmolality (UOsm>500 mOsm/kg), low urine sodium (UNa< 20 mEq/L), and fractional
excretion of sodium <1% (<2.5% in neonates) most likely have prerenal AKI. Those with
a specific gravity of <1.010, low urine osmolality (UOsm< 350 mOsm/kg), high urine
sodium (UNa> 40 mEq/L), and fractional excretion of sodium >2% (>10% in neonates),
red blood cell casts most likely have intrinsic AKl and low urine osmolality (UOsm< 350
mOsm/kg) in Post renal AKl.
72.(E). Mannitol may be effective in prevention of pigment (myoglobin, hemoglobin)
induced renal failure.
73.(B). Calcium gluconate counteracts the potassium-induced increase in myocardial
irritability but does not lower the serum potassium level.
74.(B). Hypocalcemia is primarily treated by lowering the serum phosphorus
level,phosphate binders should be orally administered to bind any ingested phosphate
and increase GI phosphate excretion. Calcium should not be given iv except in cases of
tetany to avoid deposition of calcium salts into tissues.
415
75(8). Persistent hyperkalemia.
76(0). Intermittent hemodialysis is useful in patients with relatively stable
hemodynamic status, while continuous renal replacement therapy is useful in patients
with unstable hemodynamic status, concomitant sepsis, or multiorgan failure in the
intensive care setting, hyperosmolar dialysate is infused into the peritoneal cavity via a
surgically or percutaneously placed peritoneal dialysis catheter, urea and creatinine
clearance more with intermittent hemodialysis than peritoneal dialysis.
77(D). After 5 yr of age, acquired diseases (various forms of glomerulonephritis
including lupus nephritis) and inherited disorders (familial juvenile nephronophthisis,
Alport syndrome) predominate.
78(8). Hyperkalemia is the result of CKD not a cause.
79.(C). The ultimate goal for children with ESRDis successful kidney transplantation. it
is recommended that plans for renal replacement therapy be initiated when a child
reaches stage 4 CKD.Hemodialysisis performed 3 times weekly. Peritoneal dialysis use
cycler-driven therapy reduction in the number of dialysis catheter connections and
disconnections (which decreases the risk of peritonitis).
80(0). Optimal outcomes result when the child weighs 210 kg.
81.(C).
82.(A). Owing to the high risk of developing Wilms tumor, patients with Denys-Drash
syndrome should undergo bilateral nephrectomy before transplantation.
83(C).
84(A).
85(0). infections need to be identified and treated before renal transplantation are;
Hepatitis B and Hepatitis C
86(8).
87.(A). Central to many current pediatric immunosuppressive regimens is a calcineurin
inhibitor (cyclosporine or tacrolimus) in combination with steroids and an adjunctive
anti proliferative agent (azathioprine, sirolimus, or MMF), other for induction
therapies.
88.(C). Gastrointestinal and hematologic side effects occur in mycophenolate mofetil,
but not in cyclosporine.
89(0).
90(8). That is why reducing immunosuppression is the main form of therapy, and
cidofovir and leflunomide are used as adjunctive therapies.
91(E). Schwartzformula for estimated GFR= 0.43 x height in cm/ serum creatinlne in
mg/Dl-
416
Urologic Disorders inlnfants
and Children
QUESTIONS
HTAN ALOBAlD
399!
35mL/min/1.73 m2
45 mL/min/1.73 m2
55 mL/min/1.73 m2
417
E. multicystic dysplastic kidney
for urinarytractinfectionEXCEPT
areriskfactors
6.Allthefollowing
A. male gender
B. tight clothing (underwear)
C. pinworm infestation
D. poortoilettraining
E. voiding dysfunction
D. suprapubic pain
E. urinary retention
9. According to the clinical guidelines, urine sample for diagnosis of urinary tract
infection in children 2-24 mo should be from
A. an adhesive collection bag
B. mid-stream sample
C. urine bag
D. catheterized sample
E. all of the above
418
B. cephalexin
C. ciprofloxacin
D. nitrofurantoin
E. amoxicillin
12. All the following are indicationsfor admissionto hospitalin patientswith urosepsis
EXCEPT
A. dehydration
B. unable to drink fluids
C. neonatal period
D. vomiting
E. microscopic hematuria
14. Vesicoureteral reflux is usually discovered during evaluation for a UTI, which of the
following is TRUE
autosomal dominant inheritance
males are mainly affected
16. The antenatal hydronephrosisis graded by the trimester and the antero-posterior
diameter of the renal pelvis.
419
.,, w 27' "w
03> Lizas/saw"
lS
likelycause
theMOST
Ofthefollowing,
vesicoureteralreflux
junctionobstruction
ureteropelvic
mpow? transienthydronephrosis
posteriorurethralvalve
17.Ureteropelvic
junction
junctionobstruction
ureterovesical
common
ISthemost
obstruction lesmn
obstructive _
' '
n i
childhood. .
0f the following, the MOSTcorrect statement is
A. it is usuallycausedby extrinsicstenosis
B. it mainly occurson the right side
C. there is a female preponderance
D. voiding cystourethrogram(VCUG)is necessary
E. it is usually bilateral
420
female predominance
polydramnios
W909?small bladder
cardiac abnormalities
all the above
(in 10% of cases)
22. Posterior urethral valves, is the most common cause of severe obstructive
uropathies in children.
0f the following, the MOST appropriate statement
A. its incidence 1 in 40,000 boys
B. vesicoureteral reflux occurs in 5% of patients
C. the urinary stream is strong
D. Foley catheter should be avoided
E. it is adiverticulum in the penile urethra
9.005?
normal upper urinary tracts
posteriorly displaced anus
upward displaced umbilicus is
W909?cutaneous vesicostomy
antimicrobial prophylaxis
clean intermittent catheterization
wpnw?malignant neoplasm
urinary tract infection
spontaneous perforation
26. In children up to the age of 14 yr, the mean bladder capacity in ounces is equal to
the age (in years) plus
A. 2
B. 3
421
C.
D.
E. O'iU'l#
27.The MOSTcommoncauseof daytime incontinenceis
A. an overactive bladder
B. infrequent voiding
C. detrusorsphincter dyssynergia
D. bladder outlet obstruction
E. behavioral
W909? occurrence
daytime
voiding
at 7-10 yr of age
incontinence
every 10-15 min during the day
W903? 50%
70%
90%
42
C. desmopressin acetate
D. oxybutynin chloride
E. imipramine
34. The most common causeof micropenis is failure of the hypothalamusto produce
an adequate amount of gonadotropin-releasing hormone.
0f the following, the syndrome associated with micropenis is
A. Kallmann syndrome
B. fetal hydantoin syndrome
C. Apert syndrome
D. de Lange syndrome
E. Holt-Oram syndrome
36. The MOST common male urethral anomaly associated with prune-belly syndrome is
A . congenital urethral fistula
B. urethral duplication
C. megalourethra
D. urethral hypoplasia
E. urethral atresia
38. The MOST common cause of testicular pain in a 12- yr-old boy is
423
epididymitis
testicular tumor
WP???testiculartorsion
scrotal hematoma
stone
riskofrenal
39.The
inguinalhernia
incarcerated
of
inthepresence
increases
formation
A. low urine pH
8. urinary citrate
C. urinary magnesium
D. urinarygiycosamlnoglycan
E. urinary osteopontin
424
44. Secondary hyperoxaluria can occur in patients with
A furosemide therapy
B. sarcoidosis
C. pyridoxine deficiency
D. primary hyperparathyroidism
E. immobility
46. Struvite calculi (secondary to urinary tract infections) are MOST likely caused by
A. Escherichia coli
B. Proteus
C. Klebsiella
D. Pseudomonas
E. Staph. aurous
511.0090?
increased protein diet
encourage lemon juice intake
calcium restriction
49. Which of the following drugs can precipitate and form renal stones?
A acetazolamide
B. indanivir
C. probenecid
D. theophylline
E. vitamin C
425
Urologic Disorders in Infants
and Children
ANSWERS
HTAN ALOBAID
1.(B).
2.(B). Oligohydramnios.
3.(C). Death is due to pulmonary insufficiency from pulmonary hypoplasia rather than
renal failure.
4.(E).
5.(E). Wilms tumors are 4 times more common in children with horseshoe kidneys than
in the general population, stone disease and hydronephrosis secondary to
ureteropelvic junction obstruction are other potential late complications.
6.(A). Female gender and uncircumcised male.
7.(C).
8.(D). Symptoms include dysuria, urgency, frequency, suprapubic pain, incontinence,
and malodorous urine. Cystitis does not cause fever and does not result in renal injury;
malodorous urine is not specific for a UTI.
9.(D).
10.(C). Others distracters are nonspecific markers of inflammation.
11.(D). Nitrofurantoin should not be used routinely in children with a febrile UTI
because it does not achieve significant renal tissue levels.
12.(E). Microscopic hematuria is common in acute cystitis, but micro hematuria alone
does not suggest UTI.
13.(C). The mean age at VUR resolution is 6yr.
14.(A). Idiopathic VUR appears to be an autosomal dominant inherited trait with
variable penetrance, 80% are females, primary VUR also may be discovered during
evaluation for antenatal hydronephrosis, in this select population, 80% of affected
children are male. Bladder and bowel dysfunction (constipation) may be present in 50%
of children with reflux and the average age at diagnosis is 23 yr.
15.(E). VUR occurring during bladder filling is termed low-pressure VUR; VUR during
voiding is termed high-pressure VUR.VUR in children with low-pressure VUR is
significantly less likely to resolve spontaneously than in children who exhibit only high
pressure VUR. Radiation exposure during a radionuclide cystogram is significantly less
than that from a contrast VCUG._
16.(C).
17.(D).VCUGis necessarybecause10-15%of patients have ipsilateralvesicoureteral
reflux.
18.(D).
426
19.(E).This anomaly is 3 times as common in girls as in boys.ln girls, approximately 35%
of these ureters enter the urethra at the bladder neck, 35% enter the urethra vaginal
septum, 25% enter the vagina, and a few drain into the cervix, uterus, Gartner duct, or
a urethral diverticulum. In boys, ectopic ureters enter the posterior urethra (above the
external sphincter) in 47%, the prostatic utricle in 10%, the seminal vesicle in 33%, the
ejaculatory duct in 5%, and the vas deferens in 5%.UTI is common because of urinary
stasis.
20.(C). Ureteroceles are much more common in girls than in boys. Affected children
usually are discovered by prenatal ultrasonography. Rarely, large ectopic ureteroceles
can cause bladder outlet obstruction. Ureteroceles may be ectopic, in which case the
cystic swelling extends through the bladder neck into the urethra, or orthotopic, in
which case the ureteroceles is entirely within the bladder.
21.(D). 95% of affected children are male, oligohydramnios, very large bladder.
22.(D). Foley (balloon) catheter should not be used, because the balloon can cause
severe bladder spasm, which can produce severe ureteral obstruction, affecting 1 in
8,000 boys. Vesicoureteral reflux occurs in 50% of patients and the urinary stream is
weak.The urethral valves are tissue leaflets fanning distally from the prostatic urethra
to the external urinary sphincter.
23.(C). The umbilicus is displaced downward, the anus is displaced anteriorly in both
sexes, and the wide separation of the pubic rami causes a characteristic broad-based
gait but no significant disability. In boys, there is complete epispadias with dorsal
chordee; girls also have epispadias, with separation of the 2 halves of the clitoris and
wide separation of the labia.
24.(B). Treatment includes reduction of bladder pressure with anticholinergic drugs
(e.g., oxybutynin, 0.2 mg/kg/24 hr in 2 or 3 divided doses).
25.(B). The enteric mucosal surface in contact with the urine absorbs ammonium,
chloride, and hydrogen ions and loses potassium; hyperchloremic metabolic acidosis
can result, possibly requiring medical treatment.
26.(A).
27.(A).
28.(E). In posterior urethral valves the urinary stream is weak. Hypospadias is typically
ventral deflection or severe splaying. Meatal stenosis; if the meatus is pinpoint, boys
void with a forceful, fine stream that goes a great distance. Ureteral ectopia
characterized by constant urinary dripping all day.
29.(E). This condition is termed the daytime frequency syndrome of childhood or
Pollakiuria;without dysuria, UTI, daytime incontinence, or nocturia. The most common
age for these symptoms to occur is 4-6 yr.
30.(C).
31.05).
32.(B). This form of therapy has a reported success of 30-60%, although the relapse
rate is significant, while motivational therapy; there is no evidence that this approach is
427
beneficial, Pharmacologictherapy is intended to treat the symptom of enuresis and
thusis regardedas secondline and is not curative.
33.(E). In most cases,penile development is normal, and the condition is unrecognized
until circumcision is performed or the foreskin is retractable
34.(A). Others syndromes associated with syndactyly.
35(8). Non ischemic (high-flow) priapism most commonly follows perineal trauma,
such as a straddle injury, while others are causes of ischemic (venoocclusive, low-flow)
priapism.
36.(C).
37.(B). Retractile testes may be misdiagnosed as undescended testes.
38.(C).
39.(A). Thats why treatment of renal or ureteral calculus by urinary alkalization (the
urine pH should be 26.5).lnorganic (e.g., citrate, magnesium) and organic (e.g.,
glycosaminoglycans, osteopontin) substances are known to inhibit stone formation.
Organic inhibitory compounds adsorb to the surface of the crystal, thereby inhibiting
crystal growth and nucleation.
40.(C). If the stone passes into the bladder, the child usually is asymptomatic. if the
stone is in the urethra, dysuria and difficulty voiding can result, particularly in boys.
41.(A). Others distracters are causes of calcium stones.
42.(C). This study takes only a few minutes to perform, has 96% sensitivity and
specificity in delineating the number and location of calculi, and demonstrates whether
the involved kidney is hydronephrotic or not.
43.(C). Hypercalciuria if >4 mg/kg/24 hr.
44. (C). Others distracters are causes of Hypercalciuria.
45.(E). Autosomal recessive disorder of the epithelial cells of the renal tubule that
prevents absorption of the 4 dibasic amino acids (cystine, ornithine, arginine, lysine)
and results in excessive urinary excretion of these products. The only known
complication of this familial disease is the formation of calculi, because of the low
solubility of cystine; the patients usually have acidic urine, which leads to a higher rate
of precipitation.
46.(B). Caused by urea-splitting organisms (most often Proteus spp., and occasionally
Klebsiella spp., Escherichiacoli, Pseudomonas spp., and others).
A7.(C). Hyperparathyroidism.
48(0). An excellent source of citrate (inhibitor of calcium stones) is lemonade,
reduction in dietary intake of sodium and increased potassium intake is indicated, low'
protein diets reduce urinary calcium and oxalate excretion, and calcium restriction in
children should be avoided.
49(8). All the others distractors increase the risk of stone formation but do not
{hemselves precipitate as stones.
428
E GynecologicProblemsof Childhoo
QUESTIONS
JAN ALOBAID
429
diagnostic
standard for is
puberty
precocious
investigation
The
6 gold rate
A. Tannermaturating
8. left wrist x-ray
luteinizing
C. serum levels
hormone
measurement
D. gonadotropins
E. brain MRI
ofthebreast
ofdevelopment
7.Lack andwarrants
delayed
isconsidered endocrinology
evaluationbythe ageof
A. 10 yr
B. 11 yr
C. 12 yr
D. 13 yr
E. 14 yr
8. Amastia absence
(complete with all the followingEXCEPT
ofthebreast)associated
A. Poland syndrome
B. ectodermaldysplasia
C. Crohn disease
D. congenital adrenal hypoplasia
E. hypogonadotropic hypogonadism
91.009?mastitis
lipoma
Intraductal papilloma
430
12. The MOST common tumor metastasized to the breast is
rhabdomyosarcoma
lymphoblastic leukemia
F1905?
neuroblastoma
lymphoma
cystosarcomaphyllodes
13. The imaging modality of choice for breast abnormalities in the pediatric population
is
A. mammography
B. CT scan
C. ultrasonography
D. MRI
E. PET scan
F909?cyst adenomas
teratomas
ovarian carcinoma
431
marker isused
(u-fetoproteinl Ofa"thefOHOWing
f°rthediagnosis
tumor
18The
ovariancancersEXCEPT
A. Immature teratoma
B. embryonalcarcinoma
C. mixed germ cell
D. dysgerminoma
E. endodermal sinus tumor
isthought
ofIeiomyosarcoma
19.Pathogenesis with
tobecorrelated
A. EpsteinBarrvirus
B. haemophilusinfluenzae
C. enterococcus
D. nocardia
E. Yersinia
22. sensitiveandspecificimaging
The-MOST techniqueusedfor evaluating miillerian
anomalies is
A. ultrasound
B. hysterosalpingogram
C. sonohysterography(saline-infusion s onographv)
D. MRI
E. CT scan
432
D. uterine dldelphys
E. arcuate uterus
24.Anlmperforate
hymenischaracterized EXCEPI
byallthefollowing
A. the incidenceis approximately1ln 1,000
3. normal secondarysex characters
C. more often it is diagnosedat the time of menarche
D. cannot be diagnosedin the newbornperiod
E. primary amenorrhea
GynecologicProblems of Childhoo
ANSWERS
ME).
2(8). In casesof treatment failure in H influenza or non-encapsulated H. Influenzae,
amoxicillinciavulanate is recommended.
3(8).
4(0). if diaper dermatitispersistsafter these conservative measures. or if the classic
satellite lesionsof candidaare present, treatment with an antifungai can decrease the
inflammation.
5.(C). Althoughdiagnosisis clinical,there is an association with other autoimmune or
endocrine disorders (hypothyroidism, Graves' disease, Addison disease, pernicious
anemia, and insulin~dependentdiabetes mellltus) and workup should Include
evaluation for at least thyroid dysfunction.
6.(D). Measurement of gonadotropins after gonadotropin-releasing hormone or
gonadotropin-releasinghormone-agonlst stimulation.
7(0).
8.(D). Congenitaladrenalhyperplasia
9.(E).
10(8). Physiologicswellingand tenderness occur on a cyclic basis, most commonly
during the premenstrual phase, and are secondary to hormonal stimulation and
resultingproliferativechanges.Hormonalimbalance can cause exaggerated responses
in the breast tissue,especiallyin the upper and outer quadrants. Modularity, poorly
localized tenderness,and a sorenessradiating to the axilla and arm are usual
accompanyingfindings.
11(8).
12.(A). Breasttumorsalsomay be the first manifestation of relapse (extra meduliary)in
acute lymphoblasticleukemia.
thedensebreast
13.(C).Because the visualization
obstructs
tissueoftheadolescent of
a palpablemass,mammographyis not advisedfor this age group.
14.(C).Causeaiopecia.
15(3).
16.(A).
17.(A).
18.(D). useE2,estradiol,
Indysgerminoma hCG and
chorionlcgonadotropin),
(human
asa tumor markers.
LDH(lactatedehydrogenase)
19.(A).
434
20.(E). Some products used to treat skin lesions in adults have not been approved for
children, including provider application of podophyllin resin and home application of
imiquimod, podofilox, and sinecatechins ointment.
21.(B).
22.(D). Because it can image nearly all reproductive structures, blood flow, external
contours, junctional zone resolution on T2-weighted images, and associated renal and
other anomalies. MRI also has a high correlation with surgical findings because of its
multiplanar capabilities and high spatial resolution.
23.(A).
24.(D). In the newborn period and early infancy, it may be diagnosed by a bulging
membrane caused by a mucocolpos from maternal estrogen stimulation of the vaginal
mucosa.
435
The EndocrineSystem
QUESTIONS
HAI USA
in nephrotic
ofhvp0calcemia
1.Maincauses arethefollowing
in children
syndrome
EXCEPT
decreased of renala-hydroxylase
activity
lowered serum albumin
mpnw?decreasedintestinalabsorptionof calcium
of cholecalciferol-binding
urinarylosses
increased
prednisone therapy
globulin
2. The following criteria are needed for the diagnosis of syndrome of inappropriate
secretion of antidiuretic hormone (SIADH) EXCEPT
excessive urinary sodium concentration
hyponatremia with normal serum osmolality
PROOF?
elevated urine osmolality
normal renal, adrenal, and thyroid function
absence of volume depletion
A. 0.4 U/kg/day
B. 0.5 U/kg/day
C. 0.6 U/kg/day
D. 0.7 U/kg/day
E. 1U/kg/day
W909?thyroid abscess
thyroid cyst
subacute thyroiditis
WP???sphenoid bone
ethemoid
temporal bone
bone
and inhibitinggrowthhormone(GH).
factorsplaya role in stimulating
10. Physiologic
One of the followinginhibitGH release
sleep
90??
exercise
hyperglycemia
acute illness
437
E. fasting
hormone
(CRH)
11.Corticotropin-releasing areinhibited
release
30dACTH by
A. argininevasopressin
B. oxytocin
C. angiotensinll
D. atrial natriureticpeptide
E. cholecystokinin
causeofacquired
12.TheMOSTcommon is
hypopituitarism
A. craniopharyngioma
B. eosinophilic
granuloma(histiocytosis)
C. tuberculosis
D. toxoplasmosis
E. meningitis
15. Thefollowingstimulatevasopressin
(VP)secretionEXCEPT
A. hyperosmolality
B. hypovolemla
C. hypotension
D. hyperglycemia
E nausea
A. lithium
B. vancomycin
C. amphotericin
438
D. methicillin
E. rifampin
19. Broad forehead, hypertelorism, small chin, long philtrum, camptodactyly, and fetal
finger pads are features of the following genetic overgrowth syndrome
A. Perlman syndrome
8. Sotos syndrome
C. Weaver syndrome
D. Beals syndrome
E. Beckwith-Wiedemann syndrome (BWS)
21. The MOST common brain lesion causing central precocious puberty is
A. postencephalitic scar
B. tuberculous meningitis
C. hypothalamic hamartoma
D. tuberous sclerosis
E. hydrocephalus
439
inthelst 2yrof life
mostoftenappears
maturation
andosseous
growth normal
areusually
9199!?
menarcheoccursat the expectedage
may be unilateral
often persists till puberty
WPDP?60 mU/L
80 mU/L
100 mU/L
A. estrogens
B. androgens
C. glucocorticoids
D nicotinicacid
E l-asparaginase
perma nent -
A. congenital
dyshormonogenesis hypothyroidism is
B. thyroid dysgenesis
C. Iodine
deficiency
D. defect of iodide transport
E. Pendred syndrome
30. The normal level of serum thyroid-stimulating hormone in first week of term Infants
is up to
4.4 mlU/L
9.5 mIU/L
91.0.05?
13.6 mlU/L
17.6 mlU/L
27.0 mlU/L
441
c. 5-9 pg/kg/dav
0. 8-12 pg/kg/daY
e1mwmmw
ofacquired
cause
33.TheM051common IS
hypothyrordism
A. craniopharyngioma
B. Hashimotothyroiditis
C. meningoencephalitis
D. drug-induced
E. irradiation
antibodies
34. Antithyroid or overthypothyroidism
andsubclinical
develop occurs
in
the following conditions EXCEPT
A. Down syndrome
B. Turner syndrome
C. type 1 diabetes mellitus
D. Sngren syndrome
E. Williams syndrome
9190!?
chronic hepatitis D infection
hepatitis A infection
hepatitis E infection
36. Thehrstclinicalmanifestation
of acquiredhypothyroidism
is
A. poor schoolwork
goiter
FPO?
deceleration of growth
weight gain
constipation
utinel V ' - .
hypothyroidism donein childrenWith d
suspecte
sonography
serum free T4
W909?TSH
antithyroglobulin
antiperoxidase antibodies
442
38. Chronic lymphocytic thyroiditis is the most common cause of
acquiI'Ed
hypothyroidism, with or without goiter.
All the following are true EXCEPT
A. more common in girls
8. peak incidence during adolescence
C. most of the affected childrenare
asymptomatic
D. clinical course is constant
E. familial clusters are common
40. Endemic cretinism is the most serious consequence of iodine dehciency; it includes
two different but overlapping syndromes: 3 neurologic type and a myxedematous type.
The neurologic syndrome is characterized by the following EXCEPT
intellectual disability
deaf-mutism
wpnw?disturbances in gait
patellar hyperreflexia
delayed pubertal development
41. All the following conditions are associated with Graves disease EXCEPT
type 1 diabetes mellitus
vitiligo
of
43. A painlessnodulein the thyroidor In the neckIs the usuelpresentation
childhood thyroid cancer.
is
the MOSTcommonsiteof distantmetastesls
Ofthefollowing,
443
A. mediastinum
B. long bones
C. skull
D. brain
E. lungs
iscommon
44.Hypocalcemia between
inneonates 72hroflife,
12and in
especially
infants with
birth asphyxia
sepsis
91.0090?
exchangetransfusion
hypomagnesemia
hyperbilirubinemia
of theparathyroid
or hypoplasia
45.Aplasia withthe
is oftenassociated
glands
syndrome.
DiGeorge/velocardiofacial Allthefollowing
regarding aretrue
thissyndrome
EXCEPT
A. occursin 1 in 4,000 newborns
8. causedby a deletion of chromosome22q11.2
C. hypocalcemiais transitory in the majority
D. hypocalcemiacan have its onset later in life
E. conotruncal defects of the heart occur in 75% of cases
I"???
carpopedal spasms
convulsions
delayed irregular teeth eruption
47. Laboratory
findingsin hypoparathyroidism include the following EXCEPT
A. low serumcalcium(5-7 mg/dL)
8. elevatedserumphosphorus (7-12mg/dL)
C. elevated serumalkaline
phosphatase
D. levelof 1,25(0H)203 is
usuallylow
E. normal serum magnesium
latter ischaracterized
bythe followingEXCEPT
A. medullarystenosisof the
long bones
. short stature
mpnw
delayed closure of the fontanel
advanced bone age
eye abnormalities
53.Glucocorticoids Theyincrease
playa majorrolein immuneregulation. circulating
polymorphonuclearcell
monocytes
WP???eosinophils
lymphocytes
basophils
. .
enetra tetheblood-brain andhave
barrier direct
effects
0n
rea
d"IYP
54.6lucocorttcords
thefollowing
have £XCEPT
actions
They
metabolism.
brain
brainedema
A. decrease
B. insomnia
C. increasedirritability
D. impairedmemory
abilitytoconcentrate
E. improved
with
55.Patients can
deficiency
mineralocorticoid thefollowing
develop EXCEPT
A. weightless
B. hypotension
metabolic alkalosis
won hyponatremia
hyperkalemia
cause
56.TheMOSTcommon adrenal
ofprimary in infancyis
insufficiency
A. congenitaladrenalhyperplasia
B. isolatedgiucocorticoiddeficiency
C. X-Iinked adrenal hypoplasiacongenita
D. Lemliopitz syndrome
E. adrenoleukodystrophy
446
C. chronic active hepatitis
D. type 1 diabetes mellitus
E. hypoparathyroidism
F909?10 day
14 day
21 day
F1905?
3 weeks of age
4 weeks of age
6 weeks of age
virilizedfemaleswith congenitaladrenalhyperplasia
63. Significantly (CAH)usually
undergo surgery between
2- 6 mo of age
7-12 mo of age
wgnw?
13-24 mo of age
5-9 year of age
1014 year of age
(saltwastingform of the
in congenitaladrenalhyperplasia
64. Cortisoldeficiency
disease) is treated with hydrocortisone
A. 5-10 mg/m2/24 hr
8. 10-15 mg/m2/24 hr
C. 15-20 mg/m2/24 hr
447
D. 20-25mg/m2/24hr
E. 25-30mg/m2/24hr
with
children
65.Inolder syndrome,
Cushing toobesity,
inaddition a common early
manifestation is
A purplishstriaeon abdomen
B. short stature
C. hypertension
D. hyperglycemia
E. osteoporosis
testsareofdiagnostic
66.Allthefollowing of Cushing
utilityinthediagnosis syndrome
EXCEPT
A. midnight cortisol levels
B. nighttime salivarycortisol levels
C. glucose tolerance test
D. urinary excretion of free cortisol
E. dexamethasone suppression test
P
67. In primary aldosteronism, one of the following is low
serum PH
Fifsr
!
serum carbon dioxide
serum sodium
serum chloride
serum calcium
68. Clinicalmanifestations
of pheochromocytomas
includethe following EXCEPT
A. hypertension
B. convulsions
C. pulmonary edema
D. obesity
E. good appetite
449
D. cimetidine
E. opiates
79.TheMOSTcommon
cardiac
defectinTurnersyndrome
is
aortic coarctation
isolatednonstenoticbicuspidaorticvalves
' -
E In almost 1m1'000 fema
llvebornles.
characterized
Itis bythe EXCEPT
following
A maternal meiotic
nondisjunction
isthemost
B. normalfemale
common
cause
phenotype
C. sexualdevelopment
andmenarche
are del ayed
D. talland
gangly
E. behavior disorders
450
81.The MOSTcommoncardiacdefect in Noonan
syndromeis
A aortic coarctation
B. pulmonary valvular stenosis
C. aortic stenosis
D. mitral valve prolapse
E. anomalous pulmonary venous
drainage
84. The current criteria for the diagnosis of type 1 and type 2 DM is a fasting blood
glucose that exceeds
A. 110 mg/dL
B. 115 mg/dL
c. 120 mg/dL
D. 125 mg/dL
E. 130 mg/dL
85. The clearest evidence of a role for viral infection in human type 1 DM is seen in
A. acquired rubella infection
B. live-virus rubella immunization
C. enteroviral infection
D. congenital rubella syndrome
E. mumps infection
of type 1 DM EXCEPT
86.Thefollowingfactorsare implicatedin the pathogenesis
A. prenatal influences
B. diet in infancy
C. viral infections
D. excessive exposure to certain infections
E. psychologic stress
451
lowHbA1c
87.FaIsely arenoted
levels EXCEPT
conditions
inthefollowing
A. aplasticanemia
8. pure red cell aplasia
C. blood transfusions
D. cirrhosis
treatedwitherythropoietin
E. renaldisease
forceliac
withtype1 DMhaveevidence
88.Subjects in
disease
A. 5-10%
B. 11-15%
C. 16-20%
D. 21-25%
E. 26-30%
A. <7.05
B. <7.10
C. <7.15
D. <7.20
E. <7.25
total C02 >15 mEq/L
pH >7.25
93. Cerebral edema complicating DKA remains the major cause of morbidity and
mortality in children and adolescents with T1 DM.
All the following are true EXCEPT
A. early bolus administration of insulin is a risk factor
B. high volumes of fluid is a risk factor
C. the incidence of cerebral edema in children with DKA has not changed over the
past 15-20 yr
D. radiographic imaging is frequently helpful in making the diagnosis of cerebral
edema
E. its etiology remains unknown
none? cerebrovascular
97.Guidelines T1DM
thatprepubertal
suggest
disease
peripheral vascular disease
peripheral neuropathies
patients commence
should for
screening
retinopathy
A. at diagnosis
B. 1 yr after diagnosis
453
C. 3yr after diagnosis
0. 5 yr after diagnosis
E. 10yr after diagnosis
99. Transient elevation of urinary albumin can occur in the following conditions EXCEPT
hypoglycemia
strenuous exercise
91.003?
dyslipidemia
increased waist : hip ratio
polycystic ovary syndrome
wpnw
metformin
sulfonylureas
acarbose
102. Donohuesyndromeischaracterized
bYth e -
A. intrauterine
growth EXCEPT
restriction followung
B. fasting hypoglycemia
C. acanthosis nigricans
D. postprandialhyperglycemia
E. profoundresistanceto
insulin
454
103. One of the following do not appear to be of concernin cystic fibrosis-related
diabetes (CFRD)
A. ketoacidosis
B. macrovascular complications
C. microvascular complications
D. frequent infections
E. energy needs
104. When diabetes and thyroid disease coexist, the possibility of autoimmune adrenal
insufficiency should be considered. it may be heralded by the followings EXCEPT
increasing insulin requirements
increasing pigmentation of the skin
WPOP?salt craving
asthenia
postural hypotension
105. Therapy with one of the following drugs usually results in significant insulin
resistance leading to glucose intolerance and overt diabetes
A. streptozotocin
rodenticide Vacor
PPS
high-dose oral steroid
cyclosporin
tacrolimus
455
The Endocrine System
ANSWERS
UHAIR ALM USAW
456
14.(E). Recent studies indicate that GH treatment is associated with a 6-fold increase in
the risk for type 2 diabetes and no significant increase in the risk for type 1 diabetes.
15.(D). Regulation of vasopressin (VP) secretion and serum osmolality.
Hyperosmolality, hypovolemia, and hypotension are sensed by osmosensors, volume
sensors, and barosensors, respectively. These stimulate both VP secretion and thirst.
VP, acting on the kidney, causes increased reabsorption of water (antidiuresis). Thirst
causes increased water ingestion. The results of these dual negative feedback loops
cause a reduction in hyperosmolality or in hypotension or hypovolemia, Additional
stimuli for VP secretion include nausea, hypoglycemia, and pain.
16(8). Acquired NDl can result from hypercalcemia or hypakalemia and is associated
with lithium, demeclocycline, foscarnet, clozapine, amphotericin, methicillin, and
rifampin.
17.(A). Neonates and young infants are often best treated solely with fluid therapy,
given their requirement for large volumes (3 L/m2/24 hr) of nutritive fluid.
18.(B). Cerebral salt wasting appears to be the result of hypersecretion of atrial
natriuretic peptide and is seen primarily with central nervous system disorders
including brain tumors, head trauma, hydrocephalus, neurosurgery, cerebrovascular
accidents, and brain death. Hyponatremia is accompanied by elevated urinary sodium
excretion (often >150 mEq/ L), excessive urine output, hypovolemia, normal or high uric
acid, suppressed vasopressin, and elevated atrial natriuretic peptide concentrations
(>20 pmol/L). Thus, it is distinguished from SIADH, in which normal or decreased urine
output, euvolemia, only modestly elevated urine sodium concentration, and an
elevated vasopressin level occur.
19.(C).
20(8). Precocious puberty is defined by the onset of secondary sexual characteristics
before the age of 8 yr in girls and 9 yr in boys.
21.(C). Hypothalamic hamartomas are the most common brain lesion causing central
precocious puberty.
22.lE). Breast development may regress after 2 yr, often persists for 3-5 yr, and is rarely
progressive.
23.(C).Hair appears on the mons and labia majora in girls and perineal and scrotal area
in boys; axillary hair generally appears later.
24.(E). Serum TSH levels are the most accurate test of thyroid function. Serum TSH
levels are elevated in primary hypothyroidism and suppressed in hyperthyroidism.
25.(C). At birth, there is an acute release of TSH; peak serum concentrations reach 60
mU/L 30 min following delivery in full-term infants. A rapid decline occurs in the
ensuing24 hr and a more gradual decline over the next 5 days to <10 mlU/L. The acute
increase in TSH produces a dramatic increase in levels of T4 to approximately 16 ug/dl.
and of T3 to approximately 300 ng/dL in about 4 hr. This T3 seems largely derived from
increasedperipheral conversion of T4 to T3. T4 levels gradually decrease during the lst
2 wk of life to 12 ug/dL.
457
26.(A). TBGlevelsincreasein pregnancy,in the newborn period, with hepatitis, and
with administrationof estrogens(oral contraceptives), selective estrogen receptor
modulators, heroin or methadone, mitotane, 5-fluorouracil, and perphenazine, and
they decreasewith androgens,anabolic steroids, glucocorticoids, nicotinic acid, and is
asparaginase.
27.(B).Someform of thyroid dysgenesis(apiasia,hypopiasia, or an ectopic gland) is the
most common causeof permanent congenital hypothyroidism, accounting for 8085%
of cases.
28.(C). The cord serum T4 is decreased in proportion to gestational age and
birthweight. The postnatal TSHsurge is reduced, and the more premature, veryiow~
birthweight infants with complications of prematurity, such as respiratory distress
syndrome,actuallyexperiencea decreasein serum T4 in the lst wk of life. As these
complicationsresolve,the serumT4 gradually increasesso that generally by 6 wk of life
it enters the T4 range seen in term infants.
29(0).
30(0). Thyroid-stimulatinghormonelevels:premature infants (28-36 wk); lst wk of life
0.7-27.0miU/L;term infants:birth to 4 days1.0-17.6mlU/L; 2-20 wk 0.6-5.6 mIU/L;
5 mo-20 yr 0.5-5.5mlU/L.
31.(C).Retardationof osseousdevelopmentcan be shown radiographically at birth in
approximately60%of congenitallyhypothyroid infants and indicates some deprivation
of thyroid hormone during intrauterine life. The distal femoral and proximal tibial
epiphyses,normally presentat birth, are often absent.
32.(E).The recommendedinitial starting dose is 10-15 ug/kg/day (totaling 37.5-50.0
ug/ day for most term infants).The starting dose can be tailored to the severity of
hypothyroidism.Rapidnormalizationof thyroid function has been demonstrated to be
important in achievingoptimal neurodevelopmentaloutcome.
33(8). The most commoncauseof acquired hypothyroidism is chronic lymphocytic
(Hashimoto)thyroiditis.
34.(E).Williamssyndromeis associatedwith subciinicalhypothyroidism; this does not
appearto be autoimmune,asantithyroidantibodiesare negative.
35(8). Chii with chronic hepatitis C infectionare at risk for subclinical
doesnot appear
hypothyroidilmmthis to be autoimmune,
because
antithyroid
antibodies are negative.
36.(C).Decelerationof growth is usuallythe first clinical manifestation, but this sigr1
often goes unrecognized.
37.(A).Sonographyis not indicatedunlessthere is a suspicion of a thyroid nodule on
neck palpation. in such cases,ultrasound examination is the most accurate study to
confirm the presenceof a nodule and determine if other, smaller nodules are present
in addition,an ultrasoundexaminationcan determine the nodule dimensions, textur¢
of otherfeaturesthat might influenci
or absence
(solidvscysticnature),andpresence
a decision fineneedle
to undertake aSpiration, blurred
suchasmicrocalcifications,
458
margins, taller-than-wide" shape, intranodular vascular flow, and pathologic-
appearing adjacent lymph nodes.
38.(D). The clinical course is variable. The goiter might become smaller or might
disappear spontaneously, or it might persist unchanged for years while the patient
remains euthyroid.
39.(D).
40.(E). Affected persons are goitrous but euthyroid, have normal pubertal development
and adult stature, and have little or no impaired thyroid function.
41.(C). Pernicious anemia.
42.(A). Severe liver disease, including liver failure requiring transplantation, has been
reported exclusively with propylthiouracil.
43.(E).
44.(A). Hypocalcemia is common in neonates between 12 and 72 hr of life, especially in
premature infants, in infants with asphyxia, and in infants of diabetic mothers (early
neonatal hypocalcemia).
4515). Associated abnormalities of the third and fourth pharyngeal pouches are
common; these include conotruncal defects of the heart in 25%, velopharyngeal
insufficiency in 32%, cleft palate in 9%, renal anomalies in 35%, and aplasia of the
thymus with severe immunodeficiency in 1%.
46.(B). Muscular pain and cramps are early manifestations; they progress to numbness,
stiffness, and tingling of the hands and feet.
47.(C). The serum level of alkaline phosphatase is normal or low.
48.(D). Delayed bone age.
49.(C). The 2nd metacarpal is involved least often. As a result, the index finger
occasionally is longer than the middle finger. Likewise, the 2nd metatarsal is only rarely
affected.
SO.(B). Progressive oliguria.
51.(D). The most consistent and characteristic radiographic finding is resorption of
subperiosteal bone, best seen along the margins of the phalanges of the hands.
52.(E). Distal renal tubular acidosis.
S3.(A). High doses of glucocorticoids deplete monocytes and lymphocytes, especially T
cells. They do so at least in part by inducing cell-cycle arrest in the 61 phase and by
activating apoptosis through glucocorticoid receptormediated effects. The effects on
lymphocytes are primarily exerted on T-helper 1 cells and hence on cellular immunity,
whereas the T-helper 2 cells are spared, leading to a predominantly humoral immune
response.
459
hydroxylase deficiency, almost all infants with lipoid adrenal hyperplasia, and most
infants with a deficiency of 33hydroxysteroid dehydrogenase manifest saltlosing
symptoms in the newborn period because they are unable to synthesize either cortisol
or aldosterone.
460
69.(C). Infants with Wolman disease, a rare lipid disorder caused by a deficiency of
lysosomal acid lipase, have extensive bilateral calcifications of the adrenal glands.
70.(A). Benign, hormonally inactive adrenocortical adenomas make up the majority of
incidentalomas.
71.(E).
Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) resistance
Mutations in steroid synthetic pathways
VVVVVV
Gonadal dysgenesis
Klinefelter syndrome (47,XXY)
Noonan syndrome (PTPN-11 gene mutation in many cases)
Cystic fibrosis (infertility)
are congenital causes of hypergonadotropic hypogonadism (primary hypogonadism;
testes).
72.(B).
Anorexia nervosa
Drug use
Malnutrition
VVVVVV
Chronic illness, especially Crohn disease
Hyperprolactinemia
Pituitary tumors
Pituitary infarction
Infiltrative disorders (e.g., histiocytosis, sarcoidosis)
Hemosiderosis and hemochromatosis
Radiation
are acquired causes of hypogonadotropic hypogonadism (secondary hypogonadism;
hypothalamic-pituitary).
73.(D). Verbal cognitive defects and underachievement in reading, spelling, and
mathematics are common. By late adolescence, many boys with Klinefelter syndrome
have generalized learning disabilities, most of which are language based. Despite these
difficulties, most complete high school.
74.(A).
75.(E). Weaker associations are seen with a large number of other medications and
drugs of abuse, including opiates, alcohol, and marijuana, although the association with
marijuana may not be as strong as previously thought.
76.(B). Significant gynecomastia is seen in 50% of adolescents with Klinefelter
syndrome.
77.(D). No laboratory evaluation is indicated in routine cases with no other associated
abnormality but all prepubertal cases, as well as pubertal cases with suspicious
features, should be investigated; initial laboratory evaluation should include thyroid
function tests (to rule out hyperthyroidism), testosterone, estradiol, human chorionic
gonadotropin, Iuteinizing hormone, and prolactin levels.
461
78.(A). Many patients with Turner syndrome are recognizable at birth because of a
characteristic edema of the dorsa of the hands and feet and loose skinfolds at the napg
of the neck. Low birthweight and decreased birth length are common.
79.(B). Regardless of the age, all patients with Turner syndrome at the time of diagnosis
need comprehensive cardiovascular evaluation by a cardiologist specializing in
congenital heart disease. Complete cardiologic evaluation, including echocardiography,
reveals isolated nonstenotic bicuspid aortic valves in one third to one half of patients
In later life, bicuspid aortic valve disease can progress to dilation of the aortic root.
Less-frequent defects include aortic coarctation (20%), aortic stenosis, mitral valve
prolapse, and anomalous pulmonary venous drainage.
80.(C). Sexual development and menarche are normal. Most pregnancies have resulted
in normal infants.
81.(B). The phenotype differs from Turner syndrome in several respects. Cognitive
impairment is often present, the cardiac defect is most often pulmonary valvular
stenosis or an atrial septal defect rather than an aortic defect, normal sexual
maturation usually occurs but is delayed by 2 yr on average, and premature ovarian
failure has been reported.
82.(D).
83.(B). The presentation of TZDM is typically more insidious than that with TlDM. In
contrast to patients with T1DM who are usually ill at the time of diagnosis and whose
presentation rarely spans more than a few weeks, children with TZDM often seek
medical care because of excessive weight gain and fatigue as a result of insulin
resistance and/or the incidental finding of glycosuria during routine physical
examination.
84.(D). It should be noted that a fasting blood glucose thatexceeds 125 mg/dL (6.9
mmol/L) is the accepted criterion for the diagnosis of diabetes.
85.(D). The clearest evidence of a role for viral infection in human TlDM is seen in
congenital rubella syndrome. Prenatal infection with rubella is associated with Bcell
autoimmunity in up to 70%, with development of TlDM in up to 40% of infected
children.
86.(D). A number of factors, including prenatal influences, diet in infancy, viral
infections, lack of exposureto certain infections even psychologic stress, are implicated
in the pathogenesis of TlDM, but their exact role and the mechanism by which they
trigger or aggravate autoimmunity remains uncertain.
87.(A). Falsely low HbA1c levels are noted in hemolytic anemias, pure red cell aplasiai
blood transfusions, and anemias associated with hemorrhage, cirrhosis;
myelodysplasias, or renal disease treated with erythropoietin.
88.(A). Fifteen to 30% of subjectswith TlDM have elevated thyroid-stimulating
hormone(TSH)and antithyroidantibodiesand closeto 5-10%have evidencefor celiai
disease.Thesediseasessharecommongenesand likely the same interplay betweer1
environmental and immunologic factors.
89.(B). An increased ion gap.
462
90.(C). To continue the insulin infusion without causing hypoglycemia, glucose must be
added to the infusion. We typically recommend that glucose be added as a 5% solution
when the serum glucose has decreased <300 mg/dL and as a 10% solution when the
serum glucose has decreased <200 mg/dL. The insulin infusion can also be lowered
from the initial maximal rate if, despite the above outlined interventions, the serum
glucose falls further.
91.(C).
92.(B). Any child can be easily transitioned to oral intake and subcutaneous insulin
when DKA has resolved (total C02 >15 mEq/L; pH >730; sodium stable between 135
and 145 mEq/L; no emesis). The first dose of short acting subcutaneous insulin is given
with a meal, and the insulin drip is discontinued approximately 30 min later.
93.(D). Radiographic imaging is frequently unhelpful in making the diagnosis of cerebral
edema.
94.(D). This syndrome is characterized by severe hyperglycemia (blood glucose >800
mg/dL), absence of or only slight ketosis, nonketotic acidosis, severe dehydration,
depressed sensorium or frank coma, and various neurologic signs that may include
grand mal seizures, hyperthermia, hemiparesis, and positive Babinski signs.
95.(A). Depending on the method used for determination, HbAlc values may be
spuriously elevated in thalassemia (or other conditions with elevated hemoglobin F)
and spuriously lower in sickle cell disease (or other conditions with high red blood cell
turnover).
96.(A). Complications of DM can be divided into 3 major categories: (1) microvascular
complications, Specifically, retinopathy and nephropathy; (2) macrovascular
complications, particularly accelerated coronary artery disease, cerebrovascular
disease, and peripheral vascular disease; and (3) neuropathies, both peripheral and
autonomic, affecting a variety of organs and systems. In addition, cataracts may occur
more frequently.
97.(D). Screening for retinopathy commenced after 5 yr duration in prepubertal
children, after 2 yr in pubertal children.
98.(A). Screening for thyroid disease and celiac disease commenced at diagnosis.
99.(A). Short-term hyperglycemia, strenuous exercise, urinary tract infections, marked
hypertension, heart failure, and acute febrile illness can cause transient elevation
urinary albumin excretion. There is marked dayto-day variability in albumin excretion,
so at least 2 of 3 collections done in a 3-6 mo period should show elevated levels
before microalbuminuria is diagnosed and treatment is started.
100(0).
101.(C). The most commonly used and the only FDA-approved oral agent for the
treatment of T2DM in children and adolescents is metformin. Renal function must be
assessed before starting metformin as impaired renal function has been associated
with potentially fatal lactic acidosis.
Signmcant hepatic dysfunction is also a contraindication to metformin use, although
mild elevations in liver enzymes may not be an absolute contraindication. The usual
463
doseis500mgoncedaily.Thismaybeincreased
starting to a maximumdoseof
2,000
mg/day.Abdominalsymptomsare common earlyin the courseof treatment,
butin
most casesthey will resolvewith time.
insulin
102.(c).Extreme acanthosis
resistance, abnormalities
nigricans, of theteethand
are featuresof Rabson-Mendenhall
nails,andpinealhyperplasia syndrome.
101(3). Macrovascular do notappearto be of concernin CFRD,perhaps
complications
because of the shortened life span of these patients.
104.(A). Decreasing insulin requirements.
105.(C). High-doseoral or parenteralsteroidtherapy usuallyresultsin significantinsulin
resistance leading to glucose intolerance and overt diabetes. The immunosuppressive
agents cyclosporinand tacrolimus are toxic to B cells, causing lDDM in a significant
proportion of patients treated with these agents. Their toxicity to pancreatic B cells was
1 of the factors that limited their usefulness in arresting ongoing autoimmune
destruction
of B cells.Streptozotocin
andthe rodenticideVacorare alsotoxicto B cells,
causing diabetes.
_ TheNervous
System
QUESTIONS
UHAIR ALM USAWI
1119.099?
three months of age
six months of age
nine months of age
465
diagnostic ofthefollowing
toolintheevaluation conditions
6. Cranial CT i savaluable
EXCEPT
A skull fractures
B. intracranialhemorrhages
C. acute infarcts
D. hydrocephalus
E. impendingherniation
toimaging
7. Anapproach withcutaneous
inpatients
ofthespine lesions
isindicated
in the following conditionsEXCEPT
hairy patch
subcutaneous mass or lipoma
WP???dermal sinus
coccygeal pits
scarlike lesions
10. Dandy-Walkermalformationis
characterized by the
following EXCEPT
ntricle
F905?
tonsilsthough the foramen
of the cerebellar
hypoplasia magnum
vermis
hydrocephalus
an enlarged posterior
fossa
466
11. Familial (autosomal recessive)microcephalyis characterizedby the following
EXCEPT
A. slanted forehead
B. prominent nose and ears
C. mild or borderline mental retardation
D. prominent seizures
E. surface convolutional markings of the brain
467
C. ocularproptosis
ofthemaxilla
D. hypoplasia
E. syndactyly
factors
risk
Minor seizure
offebrile
forrecurrence thefollowing
include EXCEPT
17.
febrileseizure
A. complex
B. age <1 yr
C. familyhistoryoffebrileseizures
D. male gender
E. lower serumsodium
areseveral
18.There afterfebrile
ofepilepsy
predictors thehighest
seizures, percent
ofriskfactorforsubsequent
epilepsy is
afterfebrileseizure
A. complexfebrile seizure,any type
B. fever <1 hr before febrile seizure
C. recurrent febrile seizures
D. focal complexfebrile seizure
E. family history of epilepsy
B. valproate
C. lamotrigine
D. acetazolamide
E. clonazepam
22. Benign myoclonic epilepsies are often best treated with
clonazepam
lamotrigine
POP? topiramate
valproate
benzodiazepines
24. Ricketsis a potential side effect from all the following EXCEPT
A. phenytoin
B. valproate
C. phenobarbital
D. primidone
E. carbamazepine
25. Epilepsy surgery is often used to treat refractory epilepsy of a number of etiologies
including the following EXCEPT
A. cortical dysplasia
B. tuberous sclerosis
C. polymicrogyria
D. degenerative problems
E. Sturge-Weber syndrome
27. All the following are true regarding diazepam in neonatal seizures EXCEPT
A. diazepam is highly lipophilic
B. it is cleared very quickly
C. recurrence of seizures is more than other anticonvulsants
D. it carries a risk of apnea and hypotension
469
asafirst-line
recommended
E. itiscurrently agent
inachild
iswarranted
28.Neuro-imaging inthefollowing
headache
with conditions
EXCEPT
examination
A. abnormalneurologic
B. afternoon headache
C. headachein children<6 yr old
D. brief coughheadache
inthechild
headache
E. migrainous ofmigraine
withnofamilyhistory
29. Tension-type
headaches bythe followingEXCEPT
(1TH)arecharacterized
A. diffuse in location
B. not affected by activity
C. throbbing quality
D. mild to moderate in severity
E. less frequently associated with nausea and photophobia
31. Definitetuberous
sclerosis
complex(TSC)isdiagnosedwhenat least2 majoror 1
2
majorplus minorfeaturesarepresent,
allthefollowingare minorfeatures EXCEPT
A shagreen patch
B. cerebral white matter
migration lines
C. multiple dental pits
D. gingival fibromas
E. bone cysts
470
A. its incidence is around 1: 36,000
B. fifty percent have a de novo gene mutation
C. hemangioblastoma of the spinal cord may be found
0. renal carcinoma is the most common cause of death
E. pheochromocytoma is a frequent association
35. The current antiepileptic drug (AEDs)of choice for primary generalizedtonic-clonic
seizures in children is
phenytoin
carbamazepine
W909? phenobarbital
valproate
topiramate
36. The MOST common type of cerebral palsy (CP) associated with seizures is
A. spastic hemiplegia
B. spastic quadriplegia
C. spastic diplegia
D. hypotonic CP
E. ataxic CP
471
areEEGs
often
39.How children?
inhealthy
abnormal
5%
10%
wpnPP 15%
20%
25%
common
40.TheMOST ofstatus
precipitant is
inchildren
epilepticus
A. CNS infection
8. fever
C. medication change
0. trauma
E. metabolic cause
WP???30 wk
32 wk
34 wk
of
of
of
corrected
corrected
corrected
gestational
gestational
gestational
age
age
age
472
45.Thefollowing are risk factorsfor deformationalplagiocephalyEXCEPT
female sex
firstborn child
919.09?
congenital
developmental delay
torticollis
47. In patients with febrile seizures, one of the following risk factors has the highest risk
for subsequent epilepsy
recurrent febrile seizures
fever <1 hr before febrile seizure
9.009? neurodevelopmental
family history of epilepsy
complex febrile seizures
abnormalities
48. The majority of patients who had prolonged febrile seizures and encephalopathy
after vaccination and who
had been presumed to have suffered from vaccine
encephalopathy (seizures and psychomotor regression occurring after vaccination and
presumed to be caused by it) turn out to have
A. generalized epilepsy with febrile seizures plus(GEFS+)
B. temporal lobe epilepsy secondary to mesial temporal sclerosis
C. myoclonic astatic seizures
D. Dravet syndrome
E. focal febrile seizures plus epilepsy variant
49. Higher risk of recurrence of the febrile seizure is associated with lower serum
A. sodium
potassium
{"9095
chloride
calcium
magnesium
so. A history of personality change in a patient with seizure could suggestthe f°"°Wi"8
as a cause of seizure
A. intracranial tumor
473
disease
B. degenerative
C. metabolicdisease
drugs
0. stimulants .
braindysfunction
E. congenital
ofafirstunprovoked
ontheevaluation
51.Guidelines nonfebrile thefollowing
seiZure,
inspecific
arerecommended
studies situations
clinical
A. head CT
B. head MRI
C. spinal tap
D. ECG
E. EEG
as
52.Drugtherapyshouldbebasedonthetypeof seizureandthe epilepsysyndrome
well as on other individual factors.
Of the following,the drugof first choicefor focal seizuresand epilepsiesis
A. carbamazepine
B. ethosuximide
C. valproate
D. lamotrigine
E. clobazam
474
E. 3yr
57. Sudden unexpected death in epilepsy (SUDEP) is the most common epilepsy related
mortality in patients with chronic epilepsy. All the following are risk factors EXCEPT
A. polypharmacology
8. female gender
C. age younger than 16 yr
D. long duration of epilepsy
E. frequent seizures
58. There are 5 main neonatal seizure types: subtle, clonic, tonic, spasms, and
myoclonic.
One of the following seizures is frequently not associatedwith electrographic
discharges
A. spasms
B. focal clonic
C. subtle
D. focal tonic
E. generalized myoclonic
ofAlcardi
arefeatures
60.Thefollowing EXCEPT
syndrome
A. coloboma of the iris
B. retinal lacunae
C. agenesisof the corpuscallosum
D. severe seizures
E. aminoaciduria
475
. in the "9Wb0rn
causegeneralizedconvulsions
61. Manyi . andlethargy
seizures,
eneralized rapidly
leading
' hiccups,
Prominent .
period.
of
arefeatures
tocoma
academia
A. propionic
syrup
B. maple disease
urine
hyperglycinemla
C. nonketotic ,
D. Leighdisease
adrenoleukodystrophy
E. neonatal
The
62. initial
drugused neonatal
acute
tocontrol isusually
seizures
A. diazepam
B. midazolam
C. phenobarbital
D. lorazepam
E. phenytoin
63.Intravenous isnotwidely
phenytoin seizures
acuteneonatal
usedto control
because of all the following EXCEPT
A. reduced solubility
8. severe local cutaneous reactions
C. interaction with other drugs
D. possible cardiac toxicity
E. not possible to mix with saline solutions
B. 15 min
C. 30min
D. 45min
E. 60min
476
midazolam
propofol
W909? levetiracetam
phenytoin
Phenobarbital
68. Migraine is the most frequent type of recurrent headache that is brought to the
attention of parents and primary care providers, but it remains under recognized and
undertreated, particularly in children.
Migraine is characterized by all the following EXCEPT
A. episodic attacks
B. moderate to severe in intensity
C. focal in location on the head
D. constantquality
E. may be associated with nausea and vomiting
69. The aura associated with migraine is a neurologic warning that a migraine is going
to occur.
0f the following, the LEASTcommon type of typical auras is
A. sensory aura
B. dysphasic aura
C. visual aura
D. vertigo
E. distortion
71. Café-au-Iaitmaculesare not specificfor NF-l; they may be seenin the following
EXCEPT
A. Noonan syndrome
477
ataxiatelangiectasia
Fanconianemia
["9959Gaucher disease
Hurler syndrome
associated type2NF-Z
withneurofibromatosis is
MOST
72The lesion
frequent
A. bilateralvestibularschwannomas .
B. intracranial
C. cataract
D. retinal hamartoma
E. skin plaque
73.Slow, continuous,
writhing, arecalled
movements
andinvoluntary
stereotypies
tics
wpnw? tremor
athetosis
chorea
74. A slowly progressive ataxia that involves the lower extremities to a greater degree
than the upper extremities. The Romberg test result is positive; the deep-tendon
reflexes are absent (particularly at the ankle), and the plantar response is typically
extensor (Babinski sign).
Of the following, the MOST likely cause of this ataxia is
A. ataxia-telangiectasia
B. Friedreich ataxia
C. abetalipoproteinemia
D. Roussy-Levy disease
E. Ramsay Hunt syndrome
478
E. dyskinetic CP
78. All the followingcan be used for treatment of cerebral palsy (CP)EXCEPT
benzodiazepines
baclofen
91.0.09)?
dantrolene
botulinum
hyperbaric
toxin
oxygen
79. Infants with Tay-Sachs disease (TSD) are characterized by the following EXCEPT
convulsions
blindness
POP? deafness
cherry-red
microcephaly
spots
(MS)includethe following
symptomsin pediatricmultiplesclerosis
81. Presenting
EXCEPT
A. hemiparesis
B. optic neuritis
C. ataxia
D. dysarthria
E. encephalopathy
82.TheMOST focalpresentation
common strokeis
of arterialischemic
A. hemiparesis
B. acutevisual deficit
479
deficit
C. speech
deficit
D. sensory
deficit
E. balance
common childhood
of
cause and
subarachnoid
intraparench
MOST
The
83 stroke(H5)is
hemorrhagic
moyamoya disease/syndrome
malformations
arteriovenous
WP???
thrombosis
sinovenous
cerebral
uremic
hemolytic syndrome
purpura
thrombocytopenic
idiopathic
visual
dysfunction,
cortical and
encephalopathy,are
seen
seizures in
Bilateral
84.
encephalopathy
hypoxicischemic
global
herpesencephalitis
FPPF? multiplesclerosis
studies
85.Theimaging
encephalopathy
hypertensive
hypoglycemia
normal
areusually of
disorders
stroke-like
inthefollowing
children
alternating hemiplegia
inborn errors of metabolism
WPOP?
vessel
hypertensive encephalopathy
hypoglycemia
global hypoxicischemic encephalopathy
childhood
primary oftheCNS
angiitis isusually
(SVcPACNS) 5"?"tad
asiéhSmall
arterial ischemic stroke
seizures
'
g 055Ofd'ffuse
CNS- -
87. The dia n
'"feCt'Ons ofcerebrospina'
onexamination
depends
fluid (CSF)obtained by lumbaI
reach
Puncture
(LP).Thecerebrospinal Prom"may
fluid(CSF)
in
3,000(mg/dL)
A. acutebacterial
meningitis
B. tuberculous
meningitis
C. fungal meningitis
D. amebic
(naegleria)
meningoencephalitis
480
E. subdural empyema
88. The organism causing CNS infection is never seen on direct examination of CSFin
A. tuberculous meningitis
B. acute bacterial meningitis
C. partially treated bacterial meningitis
D. fungal meningitis
E. amebic (naegleria) meningoencephalitis
FPPF?Neisseria meningitidis
Haemophilus influenzae
coagulasenegative
type b
Staphylococci
90. The CSF leukocyte count in normal healthy neonates may reach
A. 10 leukocytes/mm3
B. 15 leukocytes/mm3
C. 20 leukocytes/mm3
D. 25 leukocytes/mm3
E. 30 leukocytes/mm3
481
offever
duration
shorter
lowerCSF
protein
W905»?
are
94.Seizures
lowermortality
reduction
common
levels
lactate
CSF
lower
t
during
loss
hearing
insensorineural
of bacterial
hecourse
0.2 or
mg/kg/dose)
immediate
meningitis. therapy
lorazepam
(0.05
intravenous
includes (0.1-
diazepam
seizures
for
0.10mg/kg/dose).
of
management
. .
patients
seizures, receive
should.
thefollowing
immediate
After ofrecurrence
thelikelihood
reduce
to
anticonvulsant
A. valproicacid
B. phenytoin
C. phenobarbital
D. lorazepam
E. carbamazepine
common
MOST ofbacterial
sequelae
neurologic is
meningitis
95.The
A. recurrent seizures
8. delayin acquisitionof language
C. hearing loss
D. cognitive impairment
E. visual impairment
97. The following viruses have clinical manifestations similar to that of the
MRlle '
enteroviruses with the exception of m oresevere the
SIODS
Of rte"and
cerebral
attimes
an
absence
ofaCSF
pleocytosis
arboviruses
parechoviruses
W905»?
herpes simplex virus (HSV)type 1
rabies virus
Epstein-Barr virus
462
A. arboviruses
B. parechoviruses
C. West Nile virus (WNV)
D. rabies virus
E. Epstein-Barr virus
99.Thepredominant
organisms brainabscesses
causing inchllaien
are
A aerobic and anaerobic streptococci
B. Streptococcus pneumoniae
C. Enterococcus faecalis
D. bacteroides spp
E. Haemophilus aphrophilus
100. A brain abscess can be treated with antibiotics without surgery in the following
conditions EXCEPT
A abscess is <2 cm in diameter
B. illness is of short duration (<2 wk)
C. lesion is located in the posterior fossa
D. no signs of increased intracranial pressure
E. child is neurologically intact
FPPP? polycythemia
sickle cell disease
Fanconi anemia
"" '1
483
E. urinary urgency
104. The MOST common involved segments in transverse myelitis (TM) are in the
A. cervical region
B. thoracic region
C. lumbar region
D. Iumbo-sacral region
B. sacral region
- TheNervous
System
ANSWERS
ZUHAlR ALM USAWI
1.(D). in chronic subdural hemorrhages, the skull tends to assume a square or boxlike
shape, because the long-standing presence of fluid in the subdural space causes
enlargement of the middle fossa.
2.(A). Although not a routine component of the examination, smell can be tested
reliably as early as the 32nd wk of gestation by presenting a stimulus and observing for
an alerting response, withdrawal, or both. Care should be taken to use appropriate
stimuli, such as coffee or peppermint, as opposed to strongly aromatic substances (e.g.,
ammonia inhalants) that stimulate the trigeminal nerve. Each nostril should be tested
individually by pinching shut the opposite side.
3.(E).Thrombocytopenia with a platelet count <20 x 109/L.
4.(B). Normal CSFcontains up to S/mm3white blood cells (WBCs),and a newborn can
have as many as 15/mm3. Polymorphonuclear(PMN) cells are always abnormal in a
child, but 1-2/mm3 may be present in a normal neonate. An elevated PMN count
suggests bacterial meningitis or the early phase of aseptic meningitis. CSF
lymphocytosis can be seen in aseptic, tuberculous, or fungal meningitis; demyelinating
diseases; brain or spinal cord tumor; immunologic disorders, including collagen vascular
diseases; and chemical irritation (following myelogram, intrathecal methotrexate).
5.(A). Normal CSF contains no red blood cells (RBCs); thus, their presence indicates a
traumatic tap or a subarachnoid hemorrhage. Progressive clearing of the blood
between the first and last samples indicates a traumatic tap. Bloody CSF should be
centrifuged immediately. A clear supernatant is consistent with a bloody tap, whereas
xanthochromia (yellow color that results from the degradation of hemoglobin) suggests
a subarachnoid hemorrhage. Xanthochromia may be absent in bleeds <12 hr old,
particularly when laboratories rely on visual inspection rather than spectroscopy.
Xanthochromia can also occur in the setting of hyperbilirubinemia, carotenemia, and
markedly elevated CSF protein.
6.(C). CT is less useful for diagnosing acute infarcts in children, because radiographic
changes might not be apparent for up to 24 hr.
7.(D). imaging of the spine is not indicated in patients with simple dimples (<5 mm,
<25 mm from anal verge) or coccygeal pits.
8.(E). Generally, the lower the deformity is in the neuraxis (sacrum), the less likely is the
risk of hydrocephalus.
9.(E). The immobile, dull facies might give the incorrect impression of mental
retardation; the prognosis for normal development is excellent in most cases.
485
10.(B).Chiari malformation is the most common malformation of the posterior fossa
and hindbrain. it consistsof herniation of the cerebellar tonsils though the foramen
magnum.
11.(C). Familial (autosomal recessive) microcephaly is characterized by severe mental
retardation.
12.(A). Aqueductal stenosis, Chiari malformation, Dandy-Walker malformation, and
Klippel-Feil syndrome are causesof non-communicating hydrocephalus.
13.(E). A vein of Galen malformation can expand to become large and, because of its
midline position, obstruct the flow of CSF.
14.(C). The total volume of CSFapproximates 50 mL in an infant and 150 mL in an adult.
15.(D).
Sagittal: Dolicocephaly or scaphocephaly (boat-shaped)
Coronal: Unilateral: plagiocephaly Bilateral: brachycephaly, acrocephaly
Lambdoid= Lambdoid/occipital plagiocephaly; right side affected in 70% of cases
Multiple: Oxycephaly
16.(E). Apert syndrome is characterized by syndactyly of the 2nd, 3rd, and 4th fingers,
which may be joined to the thumb and the 5th finger.
17.(8).Age<1yr, durationof fever<24hr, and fever 38-39°Care major risk factorsfor
recurrence of febrile seizure.
18.(D). Focal complex febrile seizure has 29% risk for subsequent epilepsy.
19.03). This syndrome is usually caused by a new mutation, although rarely it is
inherited in an autosomal dominant manner. The mutated gene is located on 2q24-31
and encodes for SCN1A, the same gene mutated in GEFS+ spectrum. However, in
Dravet syndrome the mutations lead to loss of function and thus to a more severe
phenotype.
20.(B). An EEGwould not predict the future recurrence of febrile seizures or epilepsy
even if the result is abnormal.
21.(A). Absence seizures are most often initially treated with ethosuximide, which is as
effective as and less toxic than valproate and more effective than lamotrigine.
22.(D). Benign myoclonic epilepsies are often best treated with valproate, particularly
when patients have associated generalized tonic-clonic and absence seizures.
Benzodiazepines, clonazepam, lamotrigine, and topiramate are alternatives for the
treatment of benign myoclonic epilepsy.
23.(C).
24.(B).Potentialsideeffectsare ricketsfrom phenytoin, phenobarbital, primidone, and
carbamazepine (enzyme inducers that reduce 25-hyrdroxy-vitamin D level by inducing
its metabolism) and hyperammonemia from valproate.
25.(D).Epilepsysurgeryis often usedto treat refractoryepilepsyof a numberof
etiologies including cortical dysplasia,tuberous sclerosis, polymicrogyria, hypothalamic
hamartoma, and hemispheric syndromes, such as Sturge-Weber syndrome,
hemimegalencephaly, Rasmussen encephalitis,and Landau-Kleffnersyndrome. Patients
486
with intractable epilepsy resulting from metabolic or degenerative problems are not
candidates for resective epilepsy surgery.
26.(D). Hypoxic-ischemic encephalopathy is the most common cause of neonatal
seizures, accounting for 50-60% of patients. Seizures secondary to this encephalopathy
occur within 12 hr of birth.
27.(E). Because of the respiratory and blood pressure limitations and because the
intravenous preparation contains sodium benzoate and benzoic acid, it is currently not
recommended as a first-line agent.
28.(B). Neuroimaging is warranted when the neurologic examination is abnormal or
unusual neurologic features occur during the migraine; when the child has headaches
that awaken him or her from sleep or that are present on first awakening and remit
with upright posture; when the child has brief headaches that only occur with cough or
bending over; and when the child has migrainous headache with an absolutely negative
family history of migraine or its equivalent (e.g., motion sickness, cyclic vomiting).
29.(C). TlH are mild to moderate in severity, are diffuse in location, are not affected by
activity (although the patient may not feel like being active), and are nonthrobbing
'
(often described as a constant pressure).
30.(B). Cataracts are found in 60-81% of patients with NF-2.
31.(A). Shagreen patch is a major feature.
32.(E). It is important to note that not all children with facial port-wine stain have SWS.
In fact, the overall incidence of SWS has been reported to be 8-33% in those with a
port-wine stain.
33(8). Around 80% of individuals with VHL disease have an affected parent, and
around 20% have a de novo gene mutation.
34.(C). Arterial anomalies.
3S.(D). Class I evidence demonstrates that phenytoin, carbamazepine, phenobarbital,
primidone, valproate, topiramate, oxcarbazepine, and lamotrigine are effective for the
treatment of primary generalized tonic-clonic seizures. On the basis of efficacy and
tolerability, valproate and lamotrigine have emerged as the current drugs of choice for
grand mal seizures.
36.(A).
37.(D). The manifestations of increased intracranial pressure in an infant as compared
with an older child are:
Infant: increasing head circumference, delayed closure of the fontanel, suture
separation, bulging fontanel, failure to thrive, macrocephaly, setting sun sign, and shrill
cry.
Older child: Headache (especially in the early morning, awakening the child from sleep,
or association with vomiting), nausea, persistent vomiting, personality/mood changes,
lethargy, anorexia, fatigue, somnolence, diplopia as a result of sixth-nerve palsy or
third-nerve palsy with uncal herniation, and papilledema.
38.(E). Normal CSFprofile with the exception of an elevated opening pressure.
487
39(3). Approximately 10% of "normal" children have mild, nonspecific abnormalities in
backgroundactivity.
40(3). Fever/infection (36%) ,CNSinfection (5%) , medication change (20%) , trauma
(4%) , unknown (9%) , cerebrovascular (3%) , metabolic (8%) , ethanol/drug-related
(2%) , congenital (7%) , tumor (1%) , and anoxia (5%).
41.(E). Walks with 1 hand held, releases an object on command, comes when called,
and 1-2 meaningful words are milestones of 12-month-old child.
42(8). At 28 wk of corrected gestational age, a premature infant blinks in response to a
bright light, and at 32 wk, the infant maintains eye closure until the light source is
removed. A normal 37 wk infant turns the head and eyes toward a soft light, and a
term infant is able to fix on and follow a target, such as the examiner 5 face.
43(8).
44.(C). Premature closure of the sagittal suture produces a long and narrow skull, or
scaphocephaly, the most common form of craniosynostosis.
45.(A). Male sex.
46.(A). Riskfactors for recurrence of febrile seizure include
MAJOR
> Age <1 yr
> Duration of fever <24 hr
> Fever 38-39'C (100.4-102.2F)
MINOR
> Family history of febrile seizures
Family history of epilepsy
Complex febrile seizure
VVVVV Daycare
Male gender
Lower serum sodium at time of presentation
complex febrile seizures (more than 15 min duration or recurrent within 24 hr) 6%
" fever<1 hr before febrile seizure 11%
family history of epilepsy 18%
"
complex febrile seizures (focal) 29%
"
neurodevelopmental abnormalities 33%
4840). The majority of patients who had prolonged febrile seizures and
encephalopathy after vaccination and who had been presumed to have suffered from
vaccine encephalopathy(seizures and psychomotor regression occurring after
vaccination and presumed to be caused by it) turn out to have Dravet syndrome}
mutations, indicating that their disease is caused by the mutation and not secondary to
the vaccine. This has raised doubts about the very existence of the entity termed
vaccine encephalopathy.
49.(A). A low sodium level is associated with higher risk of recurrence of the febrile
seizure within the following 24 hr.
50.(A). A history of personality change or symptoms of increased intracranial pressure
can suggest an intracranial tumor. Similarly, a history of cognitive regression can
suggest a degenerative or metabolic disease. Certain medications such as stimulants or
antihistamines, particularly sedating ones, can precipitate seizures. A history of
prenatal or perinatal distress or of developmental delay can suggest etiologic
congenital or perinatal brain dysfunction.
51.(C). Spinal tap is considered in patients with suspected meningitis or encephalitis, in
children without brain swelling or papilledema, and in children in whom a history of
intracranial bleeding is suspected without evidence of such on head CT.
52.(A). In general, the drugs of first choice for focal seizures and epilepsies are
oxcarbazepine and carbamazepine; for absence seizures, ethosuximide; for juvenile
myoclonic epilepsy, valproate and lamotrigine; for Lennox-Gastaut syndrome,
clobazam, valproate, topiramate, lamotrigine, and, most recently, as add on,
rufinamide; and for infantile spasms, adrenocorticotropic hormone (ACTH).
53.(B). Side effects of valproic acid: weight gain, hyperammonemia, tremor, alopecia,
and menstrual irregularities. Serious: hepatic and pancreatic toxicity.
54.(A). The ketogenic diet is absolutely contraindicated in carnitine deficiency
(primary); carnitine palmitoyltransferase I or ll deficiency; carnitine translocase
deficiency; B-oxidation defects; medium-chain acyl dehydrogenase deficiency; long-
chain acyl dehydrogenase deficiency; short-chain acyl dehydrogenase deficiency; long-
chain 3-hydroxyacyl-coenzyme A deficiency; mediumchain 3-hydroxyacyl-coenzyme A
deficiency; pyruvate carboxylase deficiency; and porphyrias.
55.(C). Discontinuation of AEDs is usually indicated when children are free of seizures
for at least 2 yr.
56.(A). Older age of epilepsy onset.
57(8). Male gender.
58.(C). Spasms, focal clonic, focal tonic, and generalized myoclonic seizures are, as a
rule, associated with electrographic discharges (epileptic seizures), whereas motor
automatisms, the subtle, generalized tonic and multifocal myoclonic episodes are
frequently not associated with discharges and thus are thought to often represent
release phenomena with abnormal movements secondary to brain injury rather than
true epileptic seizures.
59.(D). Subtle seizures include transient eye deviations, nystagmus, blinking, mouthing,
abnormal extremity movements (rowing, swimming, bicycling, pedaling, and stepping),
and fluctuations in heart rate, hypertension episodes, and apnea. Subtle seizures occur
more commonly in premature than in full-term infants.
60.(E). Brain malformations account for 5-10% of neonatal seizure cases. An example is
Aicardi syndrome, which affects girls only and conSists of retinal lacunae, agenesis of
489
the corpus callosum, and severe seizures including subsequent infantile spasms with
hypsarrhythmia that is sometimes initially unilateral on EEG.
61.(C). Nonketotic hyperglycinemia, an intractable condition characterized by markedly
elevated plasma and CSF glycine levels, prominent hiccups, persistent generalized
seizures, and lethargy rapidly leading to coma.
62(0). The initial drug used to control acute seizures is usually lorazepam. Lorazepam
is distributed to the brain very quickly and exerts its anticonvulsant effect in <5 min. It
is not very lipophilic and does not clear out from the brain very rapidly. Its action can
last 6-24 hr. Usually, it does not cause hypotension or respiratory depression. The dose
is 0.05 mg/kg (range: 0.02-0.10 mg/kg) every 4-8 hr.
63(6). it is not possible to mix phenytoin or fosphenytoin with dextrose solutions.
64.(A).
65.(D). Lumbar puncture, comprehensive toxicologic screens, MRI, and other
laboratory tests are performed depending on clinical suspicion and need.
66.(A). Currently, the level of the evidence for refractory treatment is strongest for
midazolam and valproate, followed by propofol and pentobarbital/thiopental, followed
by levetiracetam, phenytoin/fosphenytoin, lacosamide, topiramate, and phenobarbital.
67.(E). All parents should be taught not to provide secondary gain when the episodes
occur, because this can reinforce the episodes.
68.(D). Migraine is characterized by episodic attacks that may be moderate to severe in
intensity, focal in location on the head, have a throbbing quality, and may be
associated with nausea, vomiting, light sensitivity, and sound sensitivity. Compared to
adults, pediatric migraine is shorter in duration and has a bilateral, often bifrontal,
location. Migraine can also be associated with an aura that may be typical (visual,
sensory, or dysphasic) or atypical (i.e., hemiplegic, Alice in Wonderland syndrome).
69(8). Dysphasic auras are the least-common type of typical aura and have been
described as an inability or difficulty to respond verbally.
70.(D). When the headache is mostly in the occipital area.
71.(E). Hunter syndrome.
72.(A). Bilateral vestibular schwannomas 90-95%.
73.(D).
74.(B). Friedreich ataxia is inherited as an autosomal-recessive disorder involving the
spinocerebellar tracts, dorsal columns in the spinal cord, the pyramidal tracts, and the
cerebellum and medulla. The onset of ataxia is somewhat later than in
ataxiatelangiectasia, but usually occurs before age 10 yr. The ataxia is slowly
progressive and involves the lower extremities to a greater degree than the upper
extremities. The Romberg test result is positive; the deeptendon reflexes are absent
(particularly at the ankle), and the plantar response is typically extensor (Babinski sign).
Patients develop a characteristic explosive, dysarthric speech, and nystagmus is present
in most children.
ii.
490
75.(D). Sydenham chorea (St. Vitus dance) is the most common acquired chorea of
childhood. It occursin 10-20% of patients with acute rheumatic fever, typically weeks
to months after a group A B-hemolytic streptococcal infection. Peak incidence is at age
8v9 yr, with a female predominance of 2:1.
76.(A). Prematurity, ischemia, infection, and endocrine/metabolic (e.g., thyroid) are
major causes of spastic diplasia.
77.(B). Spastic quadriplegia is the most severe form of CP because of marked motor
impairment of all extremities and the high association with intellectual disability and
seizures.
78.(E). Hyperbaric oxygen has not been shown to improve the condition of children
with CP.
79.(E). Macrocephaly becomes apparent by 1 yr of age and results from the 200-300
fold normal content of GMZ ganglioside deposited in the brain.
80(0). The symptoms of KD become evident in the lst few mo of life and include
excessive irritability and crying, unexplained episodes of hyperpyrexia, vomiting, and
difficulty feeding. In the initial stage of KD, children are often treated for colic or milk
allergy with frequent formula changes.
81.(E). Encephalopathy is less common and suggests consideration of ADEM or possibly
neuromyelitis optica (NMO).
82.(A). The most common focal presentation is hemiparesis, but acute visual, speech,
sensory, or balance deficits also occur. Children with these presentations require
urgent neuroimaging and consultation with a child neurologist, as emergency
interventions may be indicated.
83.(B). Arteriovenous malformations are the most common cause of childhood
subarachnoid and intraparenchymal HS and may occur anywhere.
84.(D). The posterior reversible leukoencephalopathy syndrome is seen in children with
hypertension, often in the context of an acute rise in blood pressure. Posterior regions
are selectively involved, possibly resulting in symptoms of bilateral cortical visual
dysfunction in addition to encephalopathy and seizures.
85.(A).
86.(B). Seizures are a hallmark of SVcPACNS, as more than 80% of children with
SVcPACNSpresent with seizures; all seizure types are seen.
87.(B). ln tuberculous meningitis, CSFprotein is too-3,000; may be higher in presence
of block, while in other options, it may reach 500.
88.(A). Acid-fast organisms almost never seen on smear. Organisms may be recovered
in culture of large volumes of CSF. Mycobacterium tuberculosis may be detected by
PCR of CSF.
89.(A).
90.(E). Normal healthy neonates may have as many as 30 leukocytes/mm3 (usually
<10), but older children without viral or bacterial meningitis have <5 leukocytes/mm3
in the CSF; in both age groups there is a predominance of lymphocytes or monocytes.
/
491
a
492
periorbital abscess,may require surgical drainage. The duration of antibiotic therapy
depends on the organism and response to treatment but is usually 46 wk.
101.(A). Increased intracranial pressure 2280 mm Hg in sedatedor obese children; 2250
mm Hg in nonobese, nonsedated children.
102(8). iron-deficiency anemia.
101(0). With the exception of the uncommon malignant glial tumors of the spinal
cord, which tend to present precipitously, intramedullary spinal cord tumors present in
a very insidious manner. Back pain related to the level of the tumor is a common
presenting complaint. It is likely that this pain will awaken the child from sleep and
improve as the day progresses.
104.(B).
493
Neuromuscular Disor eers
QUESHONS
n
L
anw
fasciculation
cardiomyopathy
intellectual impairment
mph?
electromyography
serum creatine kinase
nerve conduction study
494
0f the following, the MOST recognized feature of BMD is
A. cardiomyopathy
B. longer ambulation time
C. calf pseudohypertrophy
D. elevated creatine kinase
E. genetic defect at the Xp21.2 locus
6. Emery-Dreifuss muscular
or scapulohumeral
MuscularDystrophy(scapuloperoneal
dystrophy) is a rare X-linked recessive dystrophy.
Of the following, the MOST characteristic feature is
A. myotonia
B. facial weakness
C. pseudohypertrophy
D. intellectual impairment
E. dilated cardiomyopathy
8. All the following myopathies are characterized by proximal muscle wasting EXCEPT
A. Becker dystrophy
B. myotonic dystrophy
C. Duchenne dystrophy
D. central core myopathy
E. hypothyroidmyopathy
(LGMDs)are a heterogeneous
9. Limb-girdlemusculardystrophies groupof progressive
hereditarymusculardystrophlesthat mainlyaffect musclesof the hip and shoulder
girdles.
0f the following,the presentingcomplaintmay be
A. low back pain
cardiomyopathy
difficulty in swallowing
495
muscular
10.Thetermcongenital dystrophy allmuscular
because
ismisleading
dystrophiesaregeneticallydetermined.. ram other muscular dystrophies,
Adistinguishing ofthecongenital
feature f
dystrophies
is a high associationwith malformationsof
A. liver
B. lung
C. brain
D. bone
E. kidney
12. In periodic paralysis, all the following are precipitating factors EXCEPT
heavy fat meal
licorice ingestion
91.0.09?
emotional
hyperthyroidism
amphotericin
stress
B use
13. In spinal muscular atrophy (SMA) type 1, all the following are spared EXCEPT
heart
diaphragm
wpow?intelligence
anal sphincter
extraocular muscles
14. Muscle denervation is any loss of nerve supply regardless of the cause.
Of the following, the MOST specific clinical sign of denervation is
A. myotonia
B. weakness
C. fatigability
D. fasciculation
E. abnormal sensation
496
C. serum creatine kinase
D. molecular genetic test
E. motor nerve conduction study
18. in Guillain-Barré syndrome, the onset is gradual and progresses over days or weeks.
The maximal severity of weakness is usually reached by
A. 2 wk
B. 4 wk
C. 6 wk
D. 8 wk
E. 10 wk
POP? tachycardia
altered sensorium
vasomotor instability
497
C. 5th
D. 6th
E. 7th
498
D. tendon reflexes
E. respiratory muscles
26. Bell palsy is an acute unilateral peripheral facial nerve palsy that is not associated
with other cranial neuropathies or brainstem dysfunction.
Of the following, the MOST traditional treatment is
A. acyclovir
B. prednisone
C. laser therapy
D. physiotherapy
E. surgical decompression
27. Bell palsy usually develops abruptly about 2 wk after a systemic viral infection.
Of the following, the MOST common viral cause is
Mumps virus
Cytomegalovirus
28.
meow?EpsteinBarr
Charcot-Marie-Tooth
virus
Herpes simplex virus
Human herpes virus 6
499
E. tripping over their own feet
31.Myasthenia autoimmune
isachronic blockade.
ofneuromuscular
disease
gravis
Of the following,the MOST characteristicfeature is
A. fatigue
8. myalgia
C. fasciculation
D. sensory symptom
E. abnormal pupillary response
34. The Creatine kinase(CK)which is found in only 3 organsand may be separated into
corresponding isozymes: MM for skeletal muscle, MB for cardiac muscle, and BB for
brain.
Of the following, the CK level is characteristically elevated in
A. Myasthenia gravis
B. Guillain-Barré syndrome
C. WerdnigHoffmann disease
D. Duchenne muscular dystrophy
E. Emery-Dreifuss Muscular Dystrophy
35. Myotubularmyopathy,
maturational
arrestof fetal muscleduringthe myotubular
stage of development at 8-15 wk of gestation.
All the following are a characteristic features EXCEPT
A. high arch palate
B. cardiomyopathy
C. polyhydramnios
D. undescended testes
E. decrease fetal movement
501
Neuromuscular Disorders
ANSWERS
HALID ALAARJ
1.(B). An early Gowers sign is often evident by age 3 yr and is fully expressed by age 5
or 6 yr.
2.(C). Fasciculations of muscle, which are often best seen in the tongue, are a sign of
denervation is seen in spinal muscular atrophy.
3.(D). The diagnosis should be confirmed by blood PCR or muscle biopsy in every
case.
4.(B). Ambulation is important not only for postponing the psychologic depression but
also because scoliosis usually does not become a major complication as long as a
patient remains ambulatory, even for as little as 1 hr per day; scoliosis often becomes
rapidly progressive after confinement to a wheelchair.
5.(B). In BMD, boys remain ambulatory until late adolescence or early adult life. The
other distracters occur in both DMD and BMD.
6.(E). Also characterized by contractures of elbows and ankles that develop early, and
muscle becomes wasted in a scapulohumeroperoneal distribution. The serum CK value
is only mildly to moderately elevate.
7.(D). Cardiac involvement is usually manifested as heart block in the Purkinje
conduction system and arrhythmias (and sudden death) rather than as
cardiomyopathy, unlike most other muscular dystrophies.
8.(B). The distal distribution of muscle wasting in myotonic dystrophy is an exception to
the general rule of myopathies having proximal and neuropathies having distal
distribution patterns.
9.(A). Low back pain may be a presenting complaint because of the lordotic posture
resulting from gluteal muscle weakness. The other distracters are less likely to be
occurring.
10.(C). Brain malformations, particularly disorders of cortical development such as
lissencephaly/pachygyria and polymicrogyria, often complicated by severe epilepsy.
11.(E). Most patients with primary hyperparathyroidism develop weakness, fatigability,
fasciculation, and muscle wasting that is reversible after removal of the parathyroid
adenoma.
12.(A). Heavy carbohydrate meal.
13.(B). In severe infantile form, also known as Werdnig-Hoffmann disease or SMA type
1, there is diaphragmatic involvement, even late.
14.(D). Fasciculations of muscle, which are often best seen in the tongue, are a sign of
denervation. Sensory abnormalities indicate neuropathy. Fatigable weakness is
characteristic of neuromuscular junctional disorders. Myotonia is specific for a few
myopathies.
502
15.(D). The serum creatine kinase level may be normal but more commonly is mildly
elevated in the hundreds. Results of motor nerve conduction studies are normal,
except for mild slowing in terminal stages of the disease. EMG shows fibrillation
potentials and other signs of denervation of muscle. A secondary mitochondrial DNA
depletion is sometimes demonstrated in the muscle biopsy of infants with SMA. The
molecular genetic test of the SMN gene provides definite confirmation of the diagnosis.
16.(D). The original infection might have caused only gastrointestinal (especially
Campylobacter jejuni, but also Helicobacter pylori) or respiratory tract (especially
Mycoplasma pneumoniae) symptoms.
17.03).
18.(B). Weakness usually begins in the lower extremities and progressively involves the
trunk, the upper limbs, and, finally, the bulbar muscles, 3 pattern known as Landry
ascending paralysis. The onset is gradual and progresses over days or weeks; the
process plateaus in 1~28 days. Weakness can progress to inability or refusal to walk and
later to flaccid tetraplegia. Maximal severity of weakness is usually reached by 4 wk
after onset.
19.(A). Dysphagia and facial weakness are often impending signs of respiratory failure.
20.0)).
21.(C). Although areflexia is seen in MFS, patients do not have significant lower
extremity weakness compared with Guillain Barré syndrome.
22.(B). Muscle biopsy is not usually required for diagnosis; specimens appear normal in
early stages and show evidence of denervation atrophy in chronic stages.
23.(A). The dissociation between high CSF protein and a lack of cellular response in a
patient with an acute or subacute polyneurOpathy is diagnostic of Guillain-Barré
syndrome.
24.(E). IVIG administered for 2, 3, or 5 days. A commonly recommended protocol is
IVIG 0.4 g/kg/day for 5 consecutive days, but some studies suggest that larger doses
are more effective (lg/kg/day for 2 consecutive days) and related to improved
outcome.
25.(D). The clinical course is usually benign, and spontaneous recovery begins within 2
3 wk. The tendon reflexes are usually the last function to recover. Improvement
usually follows a gradient opposite the direction of involvement: bulbar function
recovering first, and lower extremity weakness resolving last.
26.(B). Oral prednisone (1 mg/kg/day for 1 wk, followed by a 1 wk taper) started within
the lst 3-5 days results in improved outcome and is a traditional treatment, its efficacy
confirmed in a recent long-term prospective study in the United Kingdom.
27.(D). Active or reactivation of herpes simplex or varicella-zoster virus may be the
most common cause of Bell palsy.
28.(D). The peroneal and tibial nerves are the earliest and most severely affected.
29.(B). The peroneal and tibial nerves are the earliest and most severely affected.
Children with the disorder are often described as being clumsy, falling easily, or tripping
over their own feet. Muscles of the anterior compartment of the lower legs become
503
wasted, and the legs have a characteristic stork-like contour. The muscular atrophy is
accompanied by progressive weakness of dorsiflexion of the ankle and eventual
footdrop. The process is bilateral but may be slightly asymmetric. Pes cavus deformities
invariably devel0p as a result of denervation of intrinsic foot muscles, further
destabilizing the gait.
30.(B). Atrophy of muscles of the forearms and hands is usually not as severe as that of
the lower extremities, but in advanced cases contractures of the wrists and fingers
produce a claw hand.
31.(A). Fasciculations of muscle, myalgias, and sensory symptoms do not occur.
Pupillary responses to light are preserved.
32.(B). Myasthenia gravis is one of the few neuromuscular diseases in which
electromyography (EMG) is more specifically diagnostic than a muscle biopsy. A
decremental responseis seen to repetitive nerve stimulation; the muscle potentials
diminish rapidly in amplitude until the muscle becomes refractory to further
stimulation. Anti-AChR antibodies should be assayed in the plasma but are
inconsistently demonstrated.
33.(A). In juvenile autoimmune myasthenia gravis, unilateral or bilateral but usually
asymmetrical ptosis and some degree of extraocular muscle weakness are the earliest
and most constant signs.
34.(D). Serum CK determination is not a universal screening test for neuromuscular
disease because many diseases of the motor unit are not associated with elevated
enzymes. The CK level is characteristically elevated in certain diseases, such as
Duchenne muscular dystrophy, and the magnitude of increase is characteristic for
particular diseases.
35.(B). At birth, affected infants have a thin muscle mass involving axial, limb girdle,
and distal muscles; severe generalized hypotonia,- and diffuse weakness. Respiratory
efforts may be ineffective, requiring ventilatory support. The testes are often
undescended. Facial muscles may be weak, but infants do not have the characteristic
facies of myotonic dystrophy. Ptosis may be a prominent feature. Ophthalmoplegia is
observed in a few cases. The palate may be high. Myotubular myopathy is not
associated with cardiomyopathy (mature cardiac muscle fibers normally have central
nuclei). The tongue is thin, but fasciculations are not seen. Tendon stretch reflexes are
weak or absent.
36.(C). Nemaline rod myopathy; Neonatal, infantile, and juvenile forms of the disease
are known. The neonatal form is severe and usually fatal because of respiratory failure
since birth. In the infantile form, generalized hypotonia and weakness, which can
include bulbar-innervated and respiratory muscles and a very thin muscle mass, are
characteristic. The head is dolichocephalic, and the palate high arched or even cleft.
Muscles of the jaw may be too weak to hold it closed.
37.(A). Mutations in the RYR1 gene are the cause of both central core myopathy and
malignant hyperthermia.
38.(8). Treatment might not be required, and there is gradual improvement over the
lst few months and no evidence of residual disease by 1 yr of age.
39.(D). Patients are usually severely weak and can have a flaccid tetraplegia with or
without bulbar and respiratory muscle involvement. Cerebrospinal fluid (CSF) shows no
pleocytosis and protein is variably normal or mildly elevated.
Disorders of the Eye
QU ESTIONS
HTAN ALOBAIDY
3. Children may have problems with going to sleep in a dark room (which may be
mistaken for a behavioral problem)
0f the following, the MOST likely cause is
A. dyslexia
B. diplopia
C. nyctalopia
D. amaurosis
E. amblyopia
PROP!retinoschisis
larval granulomatosis
9. Hypertropia is seen in
A. 3rd nerve palsy
8. 4th nerve palsy
C. 5th nerve palsy
D. 6th nerve palsy
E. 7th nerve palsy
507
C. Seesaw nystagmus
D. downbeat nystagmus
E. gaze-paretic nystagmus
W905?Staphylococcusepidermidis
Staphylococcus aureus
Streptococci
POP
topical antibiotic
systemic antibiotics
probing with topical anesthesia
14. All the following agents are used as prophylaxis to prevent neonatorum ophthalmia
EXCEPT
A. 0.5% erythromycin drops
8. 1% silver nitrate drops
C. povidone iodine (2% solution)
D. single dose of ceftriaxone
E. saline irrigation
18. All the following are options in the treatment of dendritic keratitis EXCEPT
trifluridine
topical ganciclovir
W905? systemic
cycloplegic
tepical
acyclovir
agent
corticosteroids
19. The MOST serious organism which can rapidly destroy stromal tissue and lead to
corneal perforation is
A. Neisseria gonorrhoea
B. Pseudomonas aeruginosa
C. Staphylococcus
D. Streptococcus
E. Yersinia
20. Several metabolic diseases produce distinctive corneal changes in childhood. Fine
opacities radiating in a whorl or fan-like pattern is seen in
cystinosis
mucopolysaccharidosis
Npow? gangliosidosis
Fabry disease
Wilson disease
21. The ectopia lentis (displacement of the lens) is often downward and fomard, and
the lens tends to be small and round in
A. Marfan syndrome
B. Homocystinuria
C. Weill-Marchesani syndrome
D. Sulfite oxidase deficiency
E. Ehlers-Danlos syndrome
23.Theriskfactors of prematurity
withretinopathy
associated arenotfully
(BOP)
retinalimmaturityat birthrepresentthe
known,but prematurityandthe associated
major factors.Contributory factorsinclude all the following EXCEPT
A. oxygenation
B. apnea
C. heart disease
D. hypercarbia
E. polycythemia
24. Retinopathy of prematurity (ROP) are classifiedinto 5 stages. The stage which is
characterized by the presence of a ridge and development of extraretinal fibrovascular
tissue is
A. stage 1
8. stage 2
C. stage 3
D. stage 4
E. stage 5
wpow? 3 yr
4 yr
5 yr
'
retinoblastomas,
bymarkedly
reduced
ramatic shift m 0f
thetreatment
useof
A. external beam radiation
B. systemic chemotherapy
C. Iasertherapy
D. cryotherapy
510
E. brachytherapy
28. Cherry-redspots(a bright to dull red spot at the center of the maculasurrounded
and accentuated by a grayish white or yellowish halo) are seen in all the following
EXCEPT
Tuberous sclerosis
Tay-Sachs disease
91.0090?
Sandhoff
Sulfatide
Niemann-Pick
disease
lipidosis
disease
F1905?
cotton-wool spots
papilledema
irregular narrowing of the arteriole
wpow
40% of cases
60% of cases
80% of cases
32. Vascular tortuosity, arterial and venous occlusions, salmon patches, refractile
deposits, pigmented lesions, arteriolarvenous anastomoses, and neovascularization
(with sea-fan formations); is retinopathy of
A. Iron deficiency anemia
B. polycythemia vera
C. leukemia
D. sickling disorders
E. B-thalassemia
511
33.Optic
neuritisisinflammation 0f the09m"9" withattendant
ordemyeHnllatlon
09th neuritisCE"
impairmentof function,allthefollowingmavcause
A. lead poisoning
B. chloramphenicol
C. vincristine
D. methylprednisolone
E. hydroxychloroquine
35. Of the following, the MOST common benign tumor of the orbit is
A. hemangioma
B rhabdomyosarcoma
C. lymphosarcoma
D. metastatic neuroblastoma
E. teratoma
B12
Disorders of the Eye
ANSWERS
HTAN ALOBAIDY
1.(C).
2.(A). The tumbling E test, in which the child indicates which direction the E is facing, is
the most widely used visual acuity test for preschool children.
3.(C). Nyctalopia, or night blindness, is vision that is defective in reduced illumination.
4.(D). The most common cause of a dilated unreactive pupil is purposeful or accidental
instillation of a cycloplegic agent, particularly atropine and related substances.
5.(C).
6.(E). Anisocoria meansinequality of the pupils. Dyscoria is abnormal shape of the pupil,
and corectopia is abnormal pupillary position. Microcoria (congenital miosis) appear as
a small pupil that does not react to light or accommodation and that dilates poorly, if at
all, with medication. The term aniridiais a misnomer because iris tissue is usually
present, although it is hypoplastic.
7.(E). Waardenburg syndrome is characterized principally by pigmentary disturbances
(usually a median white forelock and patches of hypopigmentation of the skin).
8.(A).
9.(B).
10.(A).
11.(A). The nystagmus is characteristically very fine, very rapid, horizontal, and
pendular; it is often asymmetric, sometimes unilateral. Signs usually develop within the
lst yr or 2 of life. Components of the triad may develop at various times. In many (ases,
the condition is benign and self-limited, usually lasting a few months, sometimes years.
12.(E).
13(8). Cleansing of the lids with warm water.
14.(E). The eye should be irrigated initially with saline every 10-30 min. gradually
increasing to 2-hr intervals until the purulent discharge has cleared,this is as part of
treatment but not as prevention. An infant born to a woman who has untreated
gonococcal infection should receive a single dose of ceftriaxone, 50 mg/kg (maximum
125 mg) IV or IM, in addition to topical prophylaxis.
15.03).
16.(A). Signs of keratoconus include Munson sign (bulging of the lower eyelid on
looking downward) and the presence of a Fleischer ring (a deposit of iron in the
epithelium at the base of the cone). Myogenic retraction of the upper lid occurs in
thyrotoxicosis, in which it is associated with 3 classic signs: a staring appearance
(Dalrymple sign), infrequent blinking (Stellwag sign), and lag of the upper lid on
downward gaze (von Graefe sign).
513
17.(D). A dermoid usually appears as a wellcircumscribed rounded or oval, gray or
pinkish-yellow mass with a dry surface from which short hairs may protrude. It may
effect only the superficial layers of the cornea, although full-thickness involvement is
common.
514
The Ear
QUESTIONS
ANEIN GHALI
515
6. Ofthefollowing, infectious
thecongenital pathogen '" sensorineural
implicated
hearing loss in children is
Measles virus
GDP?
Mumps virus
Streptococcus pneumoniae
. Lymphocyticchoriomeningitisvirus
E. H.influenza
7.Allthefollowing ofsudden
arecauses lossEXCEPT
hearing
sensorineural
A. autoimmune disease
B. Epstein Barr virus infection
C. thromboembolic event
D. trauma
E. Rubella virus infection
8. All the followingare includedin the normalflora of the externalear canal EXCEPT
A. Coagulase negative staphylococcus
B. Micrococcus
C. Diphtheroids
D. Pseudomonas aeruginosa
E. E. coli
trauma
W905? excessive
foreign body
wetness
previous infection
518
E. otorrhea
15. A major physical examination point to differentiate externa otitis from mastoiditis
and otitis media is
A. visualization of tympanic membrane
B pus discharge from ear canal
C. pain on manipulation of the auricle
D. periauricularlymphadenopathy
E hearing loss
517
ofswimming
avoidance theepisode
during
910.05
ear protection
topical neomycin
use of hair dryer after swimming
of otitismedia(OM)isintheageOf
18.Thepeakincidence
A. 1-2 yr of life
8. 4-5 yr of life
C. 6-7 yr of life
D. 9-11 yr of life
E. 13-15 yr of life
.mposr
tympanostomy
myringotomy
narcotic analgesics
21. An important predictor for the development of recurrent and chronic otitis media is
A. gender
race
FPO!
age
genetic background
bottle feeding
518
E. high incidence of respiratory tract infections
24. Using standard culture techniques, the pathogens typically found in acute otitis
media with effusion are recoverable in
A. 10%
B. 30%
C. 50%
D. 70%
E. 90%
25. The reason behind progressive decline in the occurrence of otitis media as children
grow older is
A. frequent exposure to the causative organisms
8. improved immune response
C. progressive reduction in eustachian tube wall compliance
D. the more-use of over-the counter medications
E. reduced incidence of respiratory tract infections
519
symptom
andspecific
sensitive otitismediais
of acute
theLEAST
28.Ofthefollowing,
A. pulling at the ear
8. pain
C. pus discharge
D. hearing loss
E. fullness in the ear
30. The MOSTcommon finding of the tympanic membrane in middle ear effusion is
A. bulging
B. impairment of mobility
C. opacification
D. white color
E. reduced translucency
31. The MOST specific hnding of the tympanic membrane in acute otitis media is
A. bulging
impairment of mobility
FPO?"
perforation
pale color
reduced translucency
520
often occurs in multiple family members
W909"
often affects infants
ineffective topical ocular antibiotics
bulging of tympanic membrane
34. "Watchful waiting" and analgesics use in otitis media treatment is the right choice
In
£71905?
a one-year-old infant having bilateral acute otitis media with otorrhea
a twelve-year-old girl having unilateral acute otitis media and fever of 39° c
an eighteen-month-old
otorrhea
boy having bilateral acute otitis media without
35. The MOST crucial aspect in the treatment of acute otitis media is
A. choosing the right antibiotic
B. skillful examination of the ear
C. culture of middle ear aspirate
D. accurate diagnosis
E. analgesics use
36. Of the following, the recommended first line treatment of acute otitis media is
A. amoxicillin
B cefdinir
C. cefuroxime
D. clindamycin
E. cefpodoxime
37. All the following are at greatest risk of harboring resistant bacteria in otitis media
EXCEPT
A. those younger than 2 years of age
B. those in regular contact with large group of children
C. those who recently have received antimicrobial treatment
D. those who have bilateral otitis media with toxicity
E. those who are immune compromised
38. Second line treatment for acute otitis media includes all the following agents
EXCEPT
521
A. trimethoprim
B. cefdinir
C. amixicillin-clavulanate
D. cefuroxime
E. ceftriaxone
39.Indications
formyringotomy withacute
inchildren media
otitis allthe
include
following EXCEPT
A. severe refractory pain
B. hyperpyrexia
C. facial paralysis
D. immune compromised
E. failure of first course of antibiotics
otitismediais
behindchronicsuppurative
40. TheMOSTcommonetiologicorganism
A. Klebseilla
Proteus
mph?
Pseudomonas
E. Coli
Candida
522
0. brain abscessthat is originatedfrom mastoiditisinvolvingthe temporal bone
E. paranasal abscess that is originated from mastoiditis
43. All the following are common organismsin all variants of acute mastoiditis EXCEPT
A. S. Pneumoniae
B. nonOtypable H. influenza
C. Pseudomonas aeruginosa
D. Klebsiella spp.
E. group A Streptococcus
meningitis
lateral sinus thrombosis
Mans»?
subdural abscess
brain abscess
facial paralysis
523
The Ear
ANSWERS
ASANEIN GHALI
1.(D).
2(0).
3(8).
4(8).
S.(C).
6.(D).
7.(E).
8.(E). The normal flora of the external canal consists mainly of aerobic bacteria and
includes Coagulase-negative staphylococci, Corynebacterium (diphtheroids),
Micrococcus, and, occasionally, Staphylococcus aureus, Viridans streptococci, and
Pseudomonas aeruginosa.
9.(C). Excessive wetness (swimming, bathing, increased environmental humidity) and
dryness (dry canal skin and lack of cerumen) are the major triggers.
10.(A). External otitis (swimmers ear) is caused most commonly by P. aeruginosa.
11.(B).
12.(D). Edema of the ear canal, erythema, and thick clumpy otorrhea are prominent
signs of the acute disease.
13.(C). Rarely, facial paralysis, other cranial nerve abnormalities, vertigo, and/or
sensorineural hearing loss are present. If these occur, necrotizing (malignant) otitis
externa is probable.
14.(A).
15.(C).
16.(C). A fungal infection of the external auditory canal, or otomycosis, is characterized
by fluffy white debris, sometimes with black spores seen; treatment includes cleaning
and application of antifungal solutions such as clotrimazole or nystatin; other
antifungal agents include m-cresyl acetate, gentian violet, and thimerosal.
17.(A). The most effective prophylaxis is instillation of dilute alcohol or acetic acid (2%)
immediately after swimming or bathing.
18,(A). The peak incidence and prevalence of OM is during the lst 2 yr of life. More
than 80% of children will have experienced at least 1 episode of OM by the age of 3 yr.
19.(C). Otitis media is a leading reason for physician visits and for use of antibiotics and
figures importantly in the differential diagnosis of fever. It often serves as the sole or
the main basis for undertaking the most frequently performed Operations in infants
and young children; myringotomy with insertion of tympanostomy tubes and
adenoidectomy, OM is also the most common cause of hearing loss in children.
"T
524
20.(A). Acetaminophen and Ibuprofen are the mainstay of pain management in acute
otitis media.
21.(C). The age of onset of OM is an important predictor of the development of
recurrent and chronic OM, with earlier age of onset having an increased risk for
exhibiting these difficulties later in life.
22.(D). Less developed immunologic defenses and less-favorable eustachian tubal
factors involving both the structure and function of the tube.
23.(C). Acute otitis media is a known complication of bronchiolitis; middle-ear aspirates
in children with bronchiolitis regularly contain bacterial pathogens, suggesting that
respiratory syncytial virus is rarely, if ever, the sole cause of their acute otitis media.
24.(B).
25.(C). Progressive reduction in tubal wall compliance with increasing age may explain
the progressive decline in the occurrence of OM as children grow older.
26.(E). In children with cleft palate, where OM is a universal finding, a main factor
underlying the chronic middle-ear inflammation appears to be impairment of the
opening mechanism of the eustachian tube.
27.(E).
28.(A).
29.(D).
30.(B).
31.(A).
32.(C).
33.(E). This is a common finding in acute otitis media regardless the causative
organism.
34.(B). Younger children, children with otorrhea, and children with bilateral acute otitis
media have a significantly enhanced benefit from antimicrobial therapy in comparison
to older children, children without otorrhea, or children with unilateral acute otitis
media.
35.(D).
364A). Amoxicillin 80-90 mg/kg/day in 2 divided doses for 10 days.
37.(O). Those who have bilateral otitis media with toxicity.
38.(A). Drugs chosen for second-line treatment should be effective against B-
Iactamaseproducing strains of non typeable H. influenzae and M. catarrhalis and
against susceptible and most non- susceptible strains of S. pneumoniae. Only 4
antimicrobial agents meet these requirements: amoxicillin-clavulanate, cefdinir,
cefuroxime axetil, and intramuscular ceftriaxone.
39.(E). Myringotomy should be considered as third-line therapy in patients that have
failed 2 courses of antibiotics for an episode of AOM.
40.(C). The most common etiologic organisms are P. aeruginosa and S. aureus;
however, the typical acute otitis media bacterial pathogens may also be the cause,
especially in younger children or in the winter months.
525
41.(B). Suppurative otitis media, paralysis of the external rectus muscle, and pam In the
ipsilateralorbit.
_ . . . .
42.(A). Neck abscess that is originated from mastord bone Infection mvolvmg the
temporal bone.
43.(D).
44.(B).
526
- TheSkin
QUESTIONS
ZUHAIR ALMUSAWI, ZAHRA ALMUSAWI
527
6.Topical have
antibiotics beenusedformany totreatlocal
years infections.
cutaneous
Ofthefollowing,
theMOST effective currently
agents
topical Is
available
A. polysporin
B. bacitracin
C. fusidic acid
0. gentamycin
E. tetracycline
7.Topicalcorticosteroids anti-inflammatory
arepotent andeffective
agents antipruritic
agents.
Medium-potencytopicalcorticosteroidsinclude
A. betamethasone
B. fluocinonide
C. halcinonide
D. clobetasol
E. hydrocortisone butyrate
-
10. Redundantskin over the posteriorpart of the
EXCEPT
n eck-IScommon
Inthefollow:
3 n
syndromes
A. Ehlers-Danlos
B. Turner
C. Noonan
D. Down
528
E. KlippeI-Feil
13. A 3-day-old neonate presented with unvaryingred purple hue restricted to left
lower limb with atrophic underlying subcutaneous tissue. The lesions become more
pronounced during changes in environmental temperature, physical activity, or crying.
Of the following, the MOST likely diagnosis is
A. reticulate capillary malformation
B. cutis marmorata telangiectatica congenital
C. harlequin color change
D. cutis marmorata
E. cutis verticis gyrate
14. A 25~day-old neonate presented with bright red, protuberant, compressible, and
sharply demarcated lesion on right cheek.
Of the following, the MOST likely diagnosisis
A. strawberry hemangioma
B. cavernous hemangioma
C. superficial infantile hemangioma
0. deep infantile hemangioma
E. tufted angioma
9199.00?
vincristine
interferon-a
propranolol
529
16. In the usualpatientwith infantilehemangiomas (IH) Wh°_h35"0 serious
and severe
complicationsor extensivegrowth resultingin tissue dEStVUCt'O"
disfigurement, treatment consists of
A. expectantobservation
B. pulsed-dye laser therapy
C. topical timolol solution
D. oral propranolol
E. oral corticosteroids
18. Evaluate for PHACES in any patient with facial hemangioma involving significant
area of face, one of the following is not a feature of PHACES
posterior fossa abnormalities
arterial cerebrovascular abnormalities
91.009?
coarctation
ear abnormalities
sternal
19. Ataxia-telangiectasia
of the aorta
abnormalities
A. xeroderma pigmentosum
8. dark complexion
C. excessive sun exposure
D. giant congenital nevus
E. immunosuppression
22. An 8-yearold female child presented with a solitary, asymptomatic, smooth, dome
shaped, blue-gray papule 8 mm in diameter on the dorsal aspect of her hand.
Of the following, the MOST likely diagnosisis
A nevus spilus
B. nevus of ota
C. Spitz nevus
D. blue nevus
E. epidermal nevus
23. The Peutz~Jeghers syndrome is characterized by melanotic macules on the lips and
mucous membranes and by gastrointestinal (GI) polyposis.
0f the following, The MOST constant feature is
diffuse hyperpigmentation of the nails
buccal mucosal macules
24. The following syndrome has a strong association with Café-au~lait spots
A. Turner syndrome
8. McCune-Albright syndrome
C. Russell-Silver syndrome
D. Noonan syndrome
E. Rubinstein-Taybi syndrome
FPO???
depigmented skin
deafness
heterochromia irides
unibrow (synophrys)
531
D. involves
haircompartmentafter
soon onset
E. usually
responsive graftlns
toautologous
27.The common
MOST intheetiology
implicated multiforme
oferythema (Em)
agent
is
Mycoplasma pneumonia
virus(HSV)labialis
Herpessimplex
FPDF?
28.Erythema
virusHSVgenitalis
Herpessimplex
Streptococcuspneumonia
epidermidis
Staphylococcus
byanabrupt,
(EM)ischaracterized
multiforme cutaneous
symmetric
eruption, MOST commonly on the
A. face
B. trunk
C. legs
D. palms and soles
E. extensor upper extremities
syndromeisdefinedasaffectedbodysurfacearea
29. Stevens-Johnson
A. <5%
3. <10%
C. <15%
D. <20%
E. <30%
30. All the following are advised in the treatment of Stevens-Johnson syndrome EXCEPT
A. topical ocular steroid
8. topiml oral anesthetics
C. systemic antibiotics for documented urinary or cutaneous infections
D. prophylactic systemic antibiotics
E. N immunoglobulin
31. Toxicepidermalnecrolysis
ischaracterized
bythe followingEXCEPT
A. widespread blister formation
B. skin tenderness
C. target lesions
. sudden onset
E. confluent erythema
532
A. epidermolysis bullosa simplex generalized (AD)
8. epidermolysis bullosa simplex-Dowling- Meara (AD)
C. junctional epidermolysis bullosaherlitz (AR)
0. dominant dystrophic epidermolysis bullosa (AD)
E. recessive dystrophic epidermolysis bullosa-hallopeau- siemens (AR)
33. Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder, best treated
initially with
A. IVIG
B methotrexate
C. azathioprine
D. cyclophosphamide
E. systemic methylprednisolone
35.
F1995? mostly on knees and elbows
usual mucous membranes
wpnwez
anticonvulsants
ampicillin
cyclosporine
captopril
533
38. Pityriasisalba occursmainly in children and causeslesionsthat are
A. hyperpigmented
B. well-defined
C. severely erythematous
D. finely scaly
E. intensely pruritic
POP? chlorothiazides
sulfonamides
barbiturates
42. The MOST common photosensitive reaction seen in children is acute sunburn,
effective treatment of the desquamative phase is with
A. topical corticosteroids
B. bland emollient
C. cool compresses
D. aloe vera products
E. calamine lotion
905?
secondary syphilis
nummular dermatitis
pityriasis rosea
534
E. pityriasis lichenoides chronica (PLC)
45. The generalized eruption of pityriasis rosea resembles a number of other diseases.
Of the following, the MOST important is
A. secondarysyphilis
B. drug eruptions
C. viral exanthems
D. guttate psoriasis
E. nummulardermatitis
535
49.A 10-year-old withanterior
child a firm,smooth,
developed
uveitis erythematous
thatgradually
papules enlarge plaques
to formannular a border
papular anda
Villth.
In Sizeonthe
normal, atrophic
slightly central centimeters
areaseveral dorsum ofthe
hands and feet.
Of the following, the MOSTlikely diagnosisis
tinea corporis
rheumatoid nodules
53. ClassicEhlers-Danlos
syndromeis characterizedb '
.
y t hefollowmgEXCEPT
A. post-termbirth
8. skin hyperelasticity
C. easy bruising
D. severe joint hypermobillty
E. subcutaneous spheroids
536
54. Urticaria pigmentosa is the most common form of mastocytosis. it can be confused
with the following EXCEPT
drug eruptions
herpes simplex
W905? pigmented
insect bites
bullous impetigo
nevi
55. The etiology is unknown in 30-50% of pediatric cases of erythema nodosum; one of
the MOST common known etiologies in children is
A. group A streptococcal infection
B. cat-scratch disease
C. leprosy
D. mycoplasma
E. brucellosis
56. A rare but potentially life threatening complication of subcutaneous fat necrosis is
A hyperkalemia
B. hypercalcemia
C. acidosis
D. septicemia
E. seizures
wPOP? inflammation
giant cells
calcium crystals
537
D. opiates
E. ciprofloxacin
60.The
following areassociated
disorders byunknown
anhidrosis
with mechanisms
EXCEPT
A. dehydration
B. uremia
C. cirrhosis
D. Addison disease
E. hypothyroidism
bromhidrosis
61.Apocrine develops asa resultoftheformation
afterpuberty of
chainfattyacidsandammonia bacteriaon short-
bytheactionof the following axullary
apocrine sweat
A. anaerobic diphtheroids
B. group A streptococcal infection
C. S.aureus
D. S.epidermidis
E. Pseudomonas aerogenosa
62. Nail pitting or grooves are usually seen in the following hair disorders
telogen effluvium
trichotillomania
538
65. Terry nails are characterized by a white ground glass appearance of the entire or
the proximal end of the nail and a normal pink distal 1-2 mm of the nail; this finding is
associated with
A. leprosy
B. tuberculosis
C. Hodgkin disease
D. arsenicpoisoning
E. hypoalbuminemia
67. Onycholysis indicates separation of the nail plate from the distal nail bed; drugs
which may cause onycholysis include
A. meronem
B. vancomycin
C. adriarnycin
D. indomethacin
E. cyclophosphamide
68. Beau lines are transverse grooves in the nail plate. They are usually indicative of
periodic trauma or episodic shutdown of the nail matrix secondary to the following
systemic diseases EXCEPT
A. mumps
B. measles
C. pneumonia
D. celiacdisease
E. hand-foot-and-mouth disease
69. Nail changes may be particularly associated with various other diseases.
The following matchings are true EXCEPT
A. psoriasis : pitting, yellow-brown discoloration, and thickening
B. lichen planus : violaceous papules in the proximal nail bed
C. Darier disease:red or white streaksthat extend longitudinally
D. alopecia areata: Transverse rows of fine pits
E acrodermatitisenteropathica:subungualparakeratoticscalingand thickening
70. Periungual fibromas that appear in late childhood should suggest a diagnosis of
A. mucous cysts
B. junctional nevi
C. Lichen planus
D. tuberous sclerosis
E. subungual exostoses
71. Angular cheilitis is characterized by inflammation and tissuring at the corners of the
mouth, often with associated erosion, maceration, and crusting.
Of the following, the LEASTlikely cause is
chapping
chronic lip lickers
FPPP? excessive
nutritional
salivation
contact dermatitis
deficiencies
to toothpaste
72. Aphthous stomatitis consists of solitary or multiple painful ulcerations occur on the
labial, buccal, lingual, sublingual, palatal, or gingival mucosa; in severe, debilitating
cases, systemic therapy with all the following may be helpful EXCEPT
A. corticosteroids
B. colchicine
C. antibiotics
D. dapsone
E. thalidomide
73. A 7-year-old child presented with painless, fluctuant, tense, 7mm, bluish papule on
the floor of the mOuth.
Of the following, the MOST likely diagnosis is
A. mucocele
B. Epstein pearls
C. Fordyce spots
D. noma
E. canker sores
540
75. Staphylococcalscalded skin syndrome is caused predominantly by phage group 2
staphylococci, particularly strains 71 and 55, which are present at localized sites of
infection.
Of the following, the MOST common focus of infection is
nasopharynx
umbilicus
76. Ecthyma resembles nonbullous impetigo in onset and appearance but gradually
evolves into a deeper, more chronic infection.
Of the following, the usual causative agent is
A. S. aureus
B. S. pneumoniae
C. H. influenzae type b
D. Clostridium perfringens
E. group A-B hemolytic streptococcus (GABHS)
77. Ecthyma gangrenosa is a necrotic ulcer covered with a gray-black eschar. It usually
occurs in immunosuppressed patients with neutropenia.
0f the following, the usual causative agent is
A. S. aureus
B. P. aeruginosa
C. S. pneumoniae
D. H. influenzae type b
E. Clostridium perfringens
541
80. All the followingmatchingare true EXCEPT
of thescalp
infection
A. Tineacapitis:dermatophyte
B. Tineacorporis:infectionof the glabrousskin
C. Tinea cruris: infection of the axilla
D. Tinea pedis: infection of the toe webs
infectionof the nailplate
E. Tineaunguium:dermatophyte
£119.55"?
primary irritant contact dermatitis
candidal diaper dermatitis
bacterial dermatitis
83. All the following can be used in the treatment of papular urticaria EXCEPT
oral antihistamines
cool compresses
ENS-709?
potent topical corticosteroids
topical antihistamines
systemic steroids
84. Scabies is transmitted only rarely by fomites because the isolated mite dies within
A. 2-3 hours
B. 12-24 hours
C. 23 days
D. 5-7 days
E. 1020 days
85. In an immunocompetent
host,scabiesis frequentlyheraldedby intensepruritus,
particularly at night.
542
Of the following, the classiclesions of scabiesare
bullae
pustules
W905? wheals
red papules
threadlike
01.005?sulfur ointment
permethrin
crotamiton
W909? malathion
lindane shampoo
benzyl alcohol lotion
88. Comedonal acne, particularly of the central face, is frequently the first sign of
pubertal maturation. It occurs in
A. 20% of adolescents
B. 40% of adolescents
C. 60% of adolescents
D. 80% of adolescents
E. 100% of adolescents
89. All the following drugs can induce acneiform lesions in susceptible individuals
EXCEPT
A. gold
B. isoniazid
C. phenytoin
D. vitamin 81
E. phenobarbital
90. Little evidence shows that ingestion of the following foods can trigger acne flares
A. high carbohydrate foods
B. high spicy foods
C. high sweet foods
D. high fatty foods
543
E. no certain foods
92. Antibiotics are indicated for treatment of patients whose acne has not responded
to topical medications.
0f the following, the antibiotic that may cause bluish discoloration of the skin and
mucous membranes is
A. tetracycline
B. doxycycline
C. minocycline
D. erythromycin
E. trimethoprim-sulfamethoxazole
F1909?topical retinoid
benzoyl peroxide
topical clindamycin
98. The replacement of elemental zinc for individuals with inherited acrodermatitis
enteropathica is equal to
A. 1 mg/kg/24 hr
B. 3 mg/kg/24 hr
C. 5 mg/kg/24 hr
D. 7 mg/kg/24 hr
E. 10 mg/kg/24 hr
99. The hair is sparse,thin, and depigmented, sometimes displaying a flag sign "which
is a characteristic finding in
A. pellagra
scurvy
PPS
vitamin a deficiency
kwashiorkor
acrodermatitis enteropathica
100. Facial eruption that frequently follows a butterfly distribution and a dermatitis
encirclingthe neck Casals necklace. is usuallyseen in
A pellagra
B. scurvy
C. vitamin A denciency
D. kwashiorkor
545
E. acrodermatitis enteropathica
1.(B).
2.(C). Nail growth is relatively slow; complete fingernail regrowth takes 6 mo, while
complete toe nail regrowth requires 12-18 mo.
3.(B). Recurrent aphthous stomatitis is present in almost all patients and is commonly
the presenting symptom.
4.(C). Medication eruptions begin on the trunk 7-10 days after exposure; they spread
peripherally and are associated with pruritus and, less commonly, with fever,
arthralgia, and lymphadenopathy. Eosinophilia may support a diagnosis of drug
eruption but may be absent in the setting of bone marrow suppression. Penicillins,
sulfa drugs, cephalosporins, nonsteroidal anti-inflammatory drugs, anticonvulsants, and
aminoglycosides are common offenders.
S.(D). Acute weeping lesions respond best to wet compresses, followed by lotions or
creams.
6.(C). Mupirocin, fusidic acid, and retapamulin are the most effective topical agents
currently available and are as effectiveas oral erythromycin in treatment of mild to
moderate impetigo.
7.(A). Corticosteroids can be divided into 7 different categories on the basis of strength,
but for practical purposes, 4 categories can be used: low, moderate, high, and super.
Low-potency preparations include hydrocortisone, desonide, and hydrocortisone
butyrate. Medium-potency compounds include amcinonide, betamethasone,
flurandrenolide, fluocinolone, mometasonefuroate, and triamcinolone. High-potency
topical steroids include fluocinonide and halcinonide. Betamethasone dipropionate and
clobetasol propionate are superpotent preparations and should be prescribed with
care.
547
13.(B).
14.(C).SuperficialiHs are bright red, protuberant, compressible, sharply demarcated
lesions that may occur on any area of the body. Although sometimes present at birth,
they more often appear in the lst 2 mo of life and are heralded by an erythematous or
blue mark or an area of pallor, which subsequently develops a fine telangiectatic
pattern before the growth phase.
15.(E).
16.(A). Expectant observation, because almost all lesions regress spontaneously,
therapy is rarely indicated. Parents require repeated reassurance and support.
17.(B). In a disfiguring, life- or vision-threatening, or ulcerated IH that is not responding
to other treatment, oral propranolol is the first-line treatment in most cases. le
typically responds with growth arrest and often early signs of involution within a couple
weeks of treatment initiation.
18.(D). PHACES stands for posterior fossa brain defects such as Dandy-Walker
malformation or cerebellar hypoplasia, large segmental facial infantile hemangioma,
arterial cerebrovascular abnormalities such as aneurysms and stroke, coarctation of the
aorta, eye abnormalities. Sternal raphe defects such as pits, scars, or supraumbilical
raphe are infrequently observed.
19.(A). The characteristic telangiectasias develop at approximately 3 yr of age, first on
the bulbar conjunctivae and later on the nasal bridge, malar areas, external ears, hard
palate, upper anterior chest, and antecubital and popliteal fossae. Additional
cutaneous Stigmata include café-au-lait spots, premature graying of the hair, and
sclerodermatous changes. Progressive cerebellar ataxia, neurologic deterioration,
sinopulmonary infections, and malignancies are also seen.
20.(E).
21(8). Fair complexion.
21(0).
23.(B). Buccal mucosal macules are the most constant feature of the disorder; in some
families, occasional members may be affected only with the pigmentary changes.
24.(B). All other distracters have weak association.
25.(A). The hallmark of Waardenburg type 1 is the white forelock, which is seen in 20
60% of patients. Only 15% of patients have areas of depigmented skin. Deafness occurs
in 9-37%, heterochromia irides in 20%, and unibrow (synophrys) in 17-69% of those
affected. Dystopia canthorum (i.e., telecanthus) is seen in all patients with
Waardenburg type 1.
26.(C). All other distracters are features of segmental type.
21(8). HSV Iabialis and, less commonly, HSV genitalis are implicated in 60-70% of
episodes of EM and are believed to trigger nearly all episodes of recurrent EM,
frequently in association with sun exposure.
28.05). Lesions are relatively sparse on the face, trunk, and legs. Lesions can be seen on
the palms and soles.
548
29.(B). 515is defined as affected body surface area <10%, SJS-TENoverlap syndrome is
affected body surface area between 10% and 30%, and TEN is affected body surface
area >30%.
30.(D). Systemic antibiotics are indicated for documentedurinary or cutaneous
infections and for suspected bacteremia (Staphylococcus aureus or Pseudomonas
aeruginosa) because infection is the leading cause of death. Prophylactic systemic
antibiotics are not necessary.
31.(C). TEN is defined by (1) widespread blister formation and morbilliform or confluent
erythema, associated with skin tenderness; (2) absence of target lesions; (3) sudden
onset and generalization within 24-48 hr; (4) histologic findings of full-thickness
epidermal necrosis and a minimal-to-absent dermal infiltrate. These criteria categorize
TEN as a separate entityfrom EM.
32.(C).
33.(E). The disease is best treated initially with systemic methylprednisolone 1-2
mg/kg/day. Azathioprine, cyclophosphamide, and methotrexate therapy all have been
useful in maintenance regimens.
34.(D). Mucous membranes are usually spared.
35.(A).
36.(B). Overwashing should be avoided because it leads to chapping and a worsening
of the dermatitis.
549
46.(A). Scaling is most prominent on the extensor aspects of the extremities,
particularly the legs. Flexural surfaces are spared, and the abdomen, neck, and face are
relatively uninvolved.
47.(A). Scaling is most pronounced on the sides of the neck, lower face, preauricular
areas, anterior trunk, and the limbs, particularly the legs.
48.(E). They may be tender or pruritic.
49.(C).
SO.(A).
51.(D). Lipoid proteinosis may be noted initially in early infancy as hoarseness. Skin
lesions appear during childhood and consist of yellowish papules and nodules that may
coalesce to form plaques. The classic sign is beaded papules on the eyelids. Lesions also
occur on the face, forearms, neck, genitals, dorsum of the fingers, and scalp, where
they result in patchy alopecia.
52.(C). Hyperelasticity and hypermobility of the joints are not present as they are in the
Ehlers-Danlos syndrome.
53.(A). Premature birth caused by rupture of membranes.
54.(B). The differential diagnosis of solitary mastocytomas includes recurrent bullous
impetigo, herpes simplex, congenital melanocytic nevi, and juvenile xanthogranuloma.
Urticaria pigmentosa can be confused with drug eruptions, postinflammatory
pigmentary change, juvenile xanthogranuloma, pigmented nevi, ephelides, xanthomas,
chronic urticaria, insect bites, and bullous impetigo. Diffuse cutaneous mastocytoma
may be confused with epidermolytic hyperkeratosis.
55.(A). Most common etiologies in children include: group A streptococcal infection,
Yersinia enterocolitica gastroenteritis, medications (cephalosporins, penicillins,
macrolides), and inflammatory disorders (inflammatory bowel disease); sarcoidosis
should be considered in young adults.
56(8). A rare but potentially life threatening complication is hypercalcemia. It
manifests at 1-6 mo of age as lethargy, poor feeding, vomiting, failure to thrive,
irritability, seizures, shortening of the QT interval on electrocardiography, or renal
failure.
57.(A). Histopathologic changes in sclerema neonatorum consist of increases in the size
of fat cells and in the width of the fibrous connective tissue septa. In contrast to SCFN,
with which this disorder is mostly to be confused, fat necrosis, inflammation, giant
cells, and calcium crystals are generally absent.
58.(D). Obstruction of sweat glands may occur in miliaria and in a number of
inflammatory and hyperkeratotic disorders, such as the ichthyoses, psoriasis, lichen
planus, pemphigus, porokeratosis, atopic dermatitis, and seborrheic dermatitis.
59.(B). Emetics.
60.(E). Diverse disorders that are associated with anhidrosis by unknown mechanisms
include dehydration; toxic overdose with lead, arsenic, thallium, fluorine, or morphine;
uremia; cirrhosis; endocrine disorders such as Addison disease, diabetes mellitus:
diabetes insipidus, and hyperthyroidism.
550
61.(A).
62.(D).
63.(E). Hypervitaminosis A.
64.(A). Large nails are seen in Pachyonychia congenita, Rubinstein-Taybi syndrome,
hemihypertrophy, while smallness or absence of nails are seen in Ectodermal
dysplasias, nail-patella, dyskeratosis congenita, focal dermal hypoplasia, cartilagehair
hypoplasia, Ellisvan Creveld, Larsen, epidermolysis bullosa, incontinentia pigmenti,
Rothmund-Thomson, Turner, popliteal web, trisomy 13, trisomy18, Apert, Gorlin-
Pindborg, long arm 21 deletion, otopalatodigital, fetal alcohol, fetal hydantoin, elfin
facies, anonychia, and acrodermatitis enteropathica.
65.(E).
66.(D). Deficient lymphatic drainage, caused by hypoplastic lymphatic vessels, is
believed to lead to the manifestations of this syndrome.
67.(D). Common causes are trauma, long-term exposure to moisture, hyperhidrosis,
cosmetics, psoriasis, fungal infection (distal onycholysis), atopic or contact dermatitis,
porphyria, drugs (bleomycin, vincristine, retinoid agents, indomethacin,
chlorpromazine [Thorazine]), and drug-induced phototoxicity from tetracyclines or
chloramphenicol.
68.(D).
69.(E). Patients with acrodermatitis enteropathica may have transverse grooves (Beau
lines) and nail dystrophy as a result of periungual dermatitis.
70.(D).
71.(D). Nutritional deficiencies are a less-frequent etiology.
72.(C). Treatment of aphthous stomatitis is palliative. The majority of mild cases do not
require therapy. Relief of pain, particularlybefore eating, may be achieved with the use
of a topical anestheticsuch as viscous lidocaine or an oral rinse with a combined
solution of elixir of diphenhydramine, viscous lidocaine, and an oral antacid.
Caution must be taken to avoid hot food and drink after topical anesthetic use. A
superpotent topical corticosteroid in a mucosa-adhering agent may help reduce
inflammation, and topical tetracycline mouthwash may also hasten healing. In severe,
debilitating cases, systemic therapy with corticosteroids, colchicine, dapsone, or
thalidomide may be helpful.
73.(A). Lesions on the floor of the mouth are known as ranulas when the sublingual or
submandibular salivary gland ducts are involved. Fluctuations in size are typical, and
the lesions may disappear temporarily after traumatic rupture. Recurrenceis prevented
by surgical excision of the mucus deposit and associated salivary gland(s).
74.(C).
75.(A). Foci of infection include the nasopharynx and, less commonly, the umbilicus,
urinary tract, a superficial abrasion, conjunctivae, and blood.
76.(E). The causative agent is usually GABHS; S. aureus is also cultured from most
lesions but is probably a secondary pathogen.
551
77.(B). Ecthyma gangrenosum occurs in up to 6% of patients with systemic P.
aeruginosa infectionbut can also occuras a primary cutaneous infection by inoculation.
78.(B). Furunculosis is also more common in individuals with low serum iron levels,
diabetes, malnutrition, HIV infection, or other immunodeficiency states.
79.(B). Vaccination with BCG characteristically produces a papule approximately 2 wk
after vaccination. The papule expands in size, typically ulcerates within 2-4 mo, and
heals slowly with scarring.
80.(C). Tinea cruris, or infection of the groin, occurs most often in adolescent males and
is usually caused by the anthropophilic species Epidermophyton floccosum or T.
rubrum, but occasionally by the zoophilic species T. mentagrophytes.
81.(D).
82.(A). They typically have a central umbilication from which a plug of cheesy material
can be expressed. The papules may occur anywhere on the body, but the face, eyelids,
neck, axillae, and thighs are sites of predilection.
83.(D). Topical antihistamines are potent immunologic sensitizers and have no role in
the treatment of insect bite reactions.
84.(C).
85.(E). Threadlike burrows are the classic lesion of scabies but may not be seen in
infants.
86.(D). The treatment of choice for scabies is permethrin 5% cream (Elimite) applied to
the entire body from the neck down, with particular attention to intensely involved
areas, which is also standard therapy.
87.(C). Because of resistance of head lice to pyrethroids, malathion 0.5% in isopropanol
is the treatment of choice for head lice and should be applied to dry hair until hair and
scalp are wet, and left on for 12 hr. A second application 7-9 days after initial treatment
may be necessary.
88.(D).
89.(D). Diagnosis of acne is rarely difficult, although flat warts, folliculitis, and other
types of acne (drug induced: glucocorticoidagents, anabolic steroids, gold,
dactinomycin, isoniazid, lithium, phenytoin, progestins) may be confused with acne
vulgaris. The differential diagnosis includes sarcoidosis, angiofibromas, keratosis pilaris,
chloracne, rosacea, and fibrofolliculomas.
90.(E). Little evidence shows that ingestion of particular foods can trigger acne flares.
When a patient is convinced that certain dietary items exacerbate acne, it is prudent
for the patient to omit those foods.
91.(C). A topical retinoid should be the primary treatment for acne vulgaris.
92.(C). Rarely, minocycline causes dizziness, intracranial hypertension, bluish
discoloration of the skin and mucous membranes, hepatitis, a lupus-like syndrome, and
drug reaction with eosinophilia and systemic symptoms.
93.(D). Topical antifungals and/or benzoyl peroxide.
94.(B). Epidermoid cysts are the nodules most commonly seen in children. Such a cyst
is a sharply circumscribed, dome-shaped, firm, freely movable, skin-colored nodule
552
often with a central dimple or punctum that is a plugged, dilated pore of a
pilosebaceousfollicle.
95.(E). Basal cell carcinoma is very rare in children in the absence of a predisposing
condition, such as nevoid basal cell carcinoma syndrome, xeroderma pigmentosum,
and nevus sebaceus of Jadassohn,arsenic intake, or exposure to irradiation. The lesions
are smooth, pearly, pink, telangiectatic papules that enlarge slowly and may bleed or
ulcerate. Sites of predilection are the face, scalp, and upper back.
96.(C).Initial signs and symptoms usually occur in the lst few mo of life, often after
weaning from breast milk to cows milk.
97.(B).
98.(B). Oral therapy with zinc compounds is the treatment of choice. Replacementfor
individuals with inherited acrodermatitis enteropathica is with 3 mg/kg/24 hr of
elemental zinc found in zinc sulfate, gluconate, or acetate (i.e., 220 mg of zinc sulfate
contains 50 mg of elemental zinc). Zinc gluconate carries less risk of gastrointestinal
distress.
99.(D). Nails are thin and soft, and hair is sparse, thin, and depigmented, sometimes
displaying a "flag sign consisting of alternating light and dark bands that reflect
alternating periods of adequate and inadequate nutrition.
100.(A).
101.(B). In children, the most common risk factors are behavioralor psychiatric disease
that results in poor nutrition.
553
Bone and Joint Disorders
QUESTIONS
A DER LMUSAWJ
C. 7-9 mo
0. 10-12 mo
E. 13-15 mo
554
D. it may be associatedwith Turner
syndrome
E. surgical removal of the extra digit is
generally performed after 2 years of age
8. In evaluation of concerns relating to the limb, the examination should assess the
exact torsional profile which include all the following EXCEPT
A. foot progression angle
8. femoral anteversion
C. knee - knee distance
D. tibial version with thighfoot angle
E. assessment of foot adduction and abduction
555
E. metatarsus adductus
withamassbehindtheknee.
agechild
11Aschool presented
medially andEhysical
dIStal
to examination
afirmmass
reveals fossa.
inthepopliteal located Knee thepoplitea'
crease. when
ismostprominent
Themass thekneeISextended. radiographs
are
normal. . -
Of the following,the MOSTlikely diagnosrsIS
A. osteochondroma
B. osteochondritis dissecans
C. popliteal cyst
D. malignancy
E. juvenile idiopathic arthritis
13. The proximal femoral ossification center (in the center of the femoral head)
appears between
A. lst and 3rd mo
8. 4th and 7th mo
C. 8th and 11th mo
D. 12th and 15th mo
E. 16th and 18th mo
14.Thereis markedgeographicandracialvariationin
the incidenceof developmental
dysplasia of the hip (DDH).
All the following are true EXCEPT
A. 1.7 in 1,000 babies in Sweden
8. 75 in 1,000 in Yugoslavia
C. 188.5in 1,000in a district in
Manitoba, Canada
D. 3.7 in 1,000 babies in Chinese
newborn
E. 0% in African newborn
557
with
D girls have
AIS asmaller
tohave
noted
been magnum
foramen
have
MS osteopema
with
ofgirls
one-third
E. approximately
thoracic
20.Thenormal spine has2050degrees asmeasured T3to
ofkyphosis
normal 0f from
valuesIs termed
T12.A thoracic in
kyphosis excessof the range
hyperkyphosis.
areassociated
conditions
Allthefollowing EXCEPT
withhyperkyphOSIs
A. trauma causingspinal fractures
B. osteogenesisimperfacta
C. Marfan syndrome
D. glycogen storage disease
E. mucopolysaccharidoses
22. Klippel-Feil syndrome includes the classic triad of a low posterior hairline, short
neck, and decreased cervical range of motion.
Other associations include the following EXCEPT
A. Sprengel's deformity
B. congenital scoliosis
C. genitourinary anomalies
D. conductive hearing loss
E. congenital heart disease
558
A. Carpenter syndrome
8. Ellis-van Creveld syndrome
C. Laurence-Moon-Biedl syndrome
D. Fanconipancytopenia
E. Trisomy 13
25. Fractures are the second most common manifestation of child abuse after skin
injury (bruises, burns/ abrasions).
All of the following fractures suggest nonaccidental injury EXCEPT
A. femur fractures in nonambulatory children
8. distal femoral metaphyseal corner fractures
C. posterior rib fractures
D. scapular spinous process fractures
E. distal humeral fractures
26. Fractures of the wrist and forearm are very common fractures in children.
All the following statements are true EXCEPT
A. the most common mechanism of injury is a fall on the outstretched hand
8. an eighty percent of forearm fractures involve the proximal radius and ulna
C. the majority of forearm fractures are torus or greenstick fractures
D. a significant malunion of a forearm diaphyseal fracture can lead to a
permanent loss of pronation and supination
E. the AP and lateral radiographs of the forearm and wrist confirm the diagnosis
27. All the following statements are true regarding clavicular fracture EXCEPT
A. most common site for fracture is the junction of the middle and medial 3rd of
clavicle
B. biceps function is important to assess as it is a prognostic indicator for future
function
C. posterior medial clavicular physeal injuries are particularly problematic
D. fractures heal rapidly usually in 36 wk
E. a palpable mass of callus is usually visible in thin children
28. Toddler fractures occur in young ambulatory children. The age range for this
fracture is typically around 14 yr.
All the following statement are true EXCEPT
A. children
in this age group are usually unable to describe the area of injury well
B. radiographs may show no fracture
C. classic symptom is refusal to bear weight
D. Inflammatory markers may be ordered to rule out infectious processes
E. fracture is treated with bed rest and analgesia for approximately 2 wk
559
forexternal
areindications
29.Allthefollowing EXCEPT
fractures
fixationinpediatric
A. grades II and Ill open fractures
B. fractures associated with severe burns
C. fractures with soft-tissue loss
D. pelvic fractures
E. fractureswith associatedhead injuriesand spasticity
30.ThetwoMOSTcommon
causative
organisms with sickle
inchildren
ofosteomyelitis
cell anemia are
A. S. aureus and streptococci
B. Salmonella spp. and S. aureus
C. S. aureus and Pseudomonas aeruginosa
D. Enterobacteriaceae and S. aureus
E. S. aureus and Bartonella henselae
@909
Pseudomonas aeruginosa
Enterobacteriaceae
Bartonella henselae
WPPF? of
andneonatesareat riskof gonococcal
adolescents
in
majority infections otherwise
septicarthritis
healthychildren
areof hematogenous
infectionof jomtsfollowingpenetratinginjuriesare uncommon
34. Whichmedicalcondition
isregardeda contraindication
origin
36. Creatine is an amino acid mostly stored in skeletal muscle increasing muscle
performance. Its use has been increased when other supplements have been
withdrawn from the market.
0f the following, the possible adverse effect include
A. mouth ulcer
B. carditis
C. hepatitis
D. nephritis
E. genital sore
561
E.V
ocular
common
40.TheM051" inMarfansyndromeis
manifestation
A. dislocationof the ocular lens
B. severe myopia
C. flat cornea
D. hypoplastic iris
E. retinal detachment
1.(B). Normal fullterm newborns can have up to 20-30 degree hip and knee flexion
contractures. These contractures tend to resolve by 4-6 mo of age.
2.(C). The ossification centers that are typically present at birth are the distal femur,
proximal tibia, calcaneus, and talus.
3.(C).The gait characteristics of a 7 yr old child are similar to those of an adult.
4.(A). Congenital vertical talus has also been described as a rocker-bottom foot or a
Persian slipper foot. The plantar surface of the foot is convex, and the talar head is
prominent along the medial border of the midfoot. The fore part of the foot is
dorsiflexed and abducted relative to the hindfoot, and the hindfoot is in equinus and
valgus.
S.(C). It is seen in approximately 2 in 1,000 births and is bilateral in 50% of cases.
Conditions that may be associated with polydactyly include Ellis-Van Creveld
(chondroectodermal dysplasia), longitudinal deficiency of the tibia, and Down
syndrome. Treatment is indicated for cosmoses and to allow for fitting with standard
shoes. This involves surgical removal of the extra digit, and the procedure is generally
performed between 9 and 12 mo of age.
5.(E).
7.(D). The normal tibiofemoral angle at birth is 10-15 degrees of physiologic varus. The
alignment changes to 0 degrees by 18 mo, and physiologic valgus up to 12 degrees is
reached in between 3 and 4 yr of age. The normal valgus of 7 degrees is achieved by 5-
8 yr of age. Persistence of varus beyond 2 yr of age may be pathologic and is seen in
conditions such as Blount disease. Overall, 95% of developmental physiologic genu
varum and genu valgum cases resolve with growth.
8.(C).
940). It occurs more commonly in girls than boys (2: 1) in children 3-6 yr of age. The
patellas are pointing inward when the foot is straight, and compensatory external
rotation of the tibia is demonstrated. Diagnosis is made clinically on examination; CT
can provide objective measurements but is rarely indicated. The torsion usually
corrects with growth by 8-10 yr of age. Persistent deformity, unacceptable cosmesis,
functional impairment, anteversion >45degrees, and no external rotation beyond
neutral are some of the indications for operative intervention.
10(8). External femoral torsion can follow a slipped capital femoral epiphysis. The
disorder is associated with an out-toeing gait and increased incidence of degenerative
arthritis. lf slipped capital femoral epiphysis is detected, it is treated surgically.
11.(C). Popliteal cysts, or Baker cysts, are cystic masses filled with gelatinous material
that develop in the popliteal fossa, the shallow depression located at the posterior part
563
of the knee. They are considered rare in children. They most commonly occur in the
region of the medial head of the gastrocnemius and semimembranosus.
Transillumination of the cyst on physical examination is a simple diagnostic test. Knee
radiographs are normal and should be obtained to identify other lesions, such as
osteochondromas, osteochondritis dissecans, and malignancies. The diagnosis may be
confirmed by ultrasonography, MRI, or aspiration.
12.(D). Patellofemoral pain syndrome (PFPS) is one of the most common causes of knee
pain, particularly in adolescent girls. The precise etiology of the knee pain remains
unknown and is likely multifactorial.
13.(B). Between the 4th and 7th mo of life, the proximal femoral ossification center (in
the center of the femoral head) appears.
14.(D). The incidence of DDH in Chinese and African newborns is almost 0%, whereas it
is 1% for hip dysplasia and 0.1% for hip dislocation in white newborns. These
differences may be the result of environmental factors, such as child-rearing practices,
rather than to genetic predisposition.
15.(A). As the baby enters the 2nd and 3rd mo of life, the soft tissues begin to tighten
and the Ortolani and Barlow tests are no longer reliable. Limitation of abduction is the
most reliable sign of a dislocated hip in this age group.
16.(E). They are often afebrile or have a low-grade fever <38°C (100.4F). The hip is not
held flexed, abducted, or laterally rotated unless a significant effusion is present.
17.(A). The most common presenting symptom is a limp of varying duration.
18.(C). Differential diagnosis of legg-calvéperthes disease include: Sickle cell disease,
other hemoglobinopathies (e.g., thalassemia), chronic myelogenous leukemia, steroid
medication, sequela of traumatic hip dislocation, treatment of developmental dysplasia
of the hip, septic arthritis, mucopolysaccharidoses, and hypothyroidism.
19.(E). AIS is 2-10 times more common in females than males. Fathers with AIS transmit
the disease to 80% of their children, but mothers with AIS transmit the disease to only
56% of their children. Lower plasma melatonin levels have been noted in patients with
progressive curvatures. Girls with MS have been noted to have a larger foramen
magnum.
20.(D). Conditions associated with hyperkyphosis are: Trauma causing spinal fractures,
spinal infections resulting from bacterial, tuberculosis, and fungal diseases, metabolic
diseases such as osteogenesis imperfecta or osteoporosis, iatrogenic (Iaminectomy,
spinal irradiation), neuromuscular diseases, neoplasms, congenital/developmental,
disorders of collagen such as Marfan syndrome, dysplasias such as neurofibromatosis,
achondroplasia, and mucopolysaccharidoses.
21.(D). Posterior fossa brain tumor.
22.(D). Other associations include Sprengels deformity (congenital elevation of the
scapula), congenital scoliosis, genitourinary anomalies (25-35%), sensorineural hearing
loss (5%), and congenital heart disease (5-10%).
23.(C). Trisomy 13
2MB). Ellis-van Creveld syndrome associated with polydactyly.
564
25.(E).Proximal humeral fractures.
26.(8). Eighty percent of forearm fractures involve the distal radius and ulna, 15%
involve the middle third, and the rest are rare fractures of the proximal third of the
radius or ulnar shaft.
27.(A).The most common site for fracture is the junction of the middle and lateral 3rd
of clavicle.
28.(E).The fracture is treated with an above-knee cast for approximately 3 wk.
29.(D). Not all pelvic fractures only the unstable one.
30.(B). Streptococcus pneumoniae most commonly causes osteomyelitis in children
younger than 24 mo of age and in children with sickle cell anemia, but its frequency has
declined because of pneumococcal conjugate vaccines. Cases of Pseudomonas
infection are related almost exclusively to puncture wounds of the foot, with direct
inoculation of P. aeruginosa from the foam padding of the shoe into bone or cartilage,
which develops as osteochondritis. Bartonella henselae can cause osteomyelitis of any
bone, but especially in pelvic and vertebral bones.
31.(B). Kingella kingae may be the second most common cause of osteomyelitis in
children younger than 5 yr of age in some parts of the world. The organism is
increasingly recognized as a cause of osteomyelitis, spondylodiskitis, and septic
arthritis, especially when polymerase chain reaction testing is employed.
32.(A). Femur 23-28%, tibia 20-26%, humerus 8-13%, radius and ulna 56%.
33.(A). Half of all cases occur by 2 yr of age and three-fourths of all cases occur by 5 yr
of age.
34.(C). Carditis can result in sudden death with exertion. All the other conditions need
individual assessment.
35.(D). Hypertension.
36.(D). Concerns about nephritis in case reports have not been supported by controlled
studies.
31(8). injuries of the shoulder in competitive swimming are most common and
generally a result of chronic, overuse. Swimmer's shoulder is a combination of
subacromial bursitis and tendinosis of the rotator cuff and longhead of the biceps.
38.(A). l: Mild, Nondeforming, ll: Lethal perinatal, JIll: Progressively deforming, IV:
Moderately deforming
39.(C). Birthweight and length are often low normal. Fractures usually occur in utero.
There is relative macrocephaly and triangular facies. Postnatally, fractures occur from
inconsequential trauma and heal with deformity. Disorganization of the bone matrix
results in a "popcorn appearance at the metaphyses. The rib cage has flaring at the
base, and pectal deformity is frequent. Virtually all type III patients have scoliosis and
vertebral compression. Growth falls below the curve by the lst yr; all type III patients
have extreme short stature. Scleral hue ranges from white to blue. Dentinogenesis
imperfecta, hearing loss, and kyphoscoliosis may be present or develop over time.
40.(A). Dislocation of the ocular lens (ectopia lentis) occurs in approximately 60-70% of
patients, although it is not unique to the disorder.
565
41.(B). B-Blockers have traditionally been consideredthe standard of care in MFS and
multiple small observational studies suggest there is a protective effect on aortic root
growth, with the dose typically titrated to achieve a resting heart rate <100 beats/min
during submaximal exercise.
Rehabilitation Medicine
QUESTIONS
UHAIR ALMUSAWI
2. Osteopenia begins immediately after a spinal cord injury (SCI) occurs and plateaus 6-
12 mo later. Pathologic fractures occur as a consequence of loss of bone mineral
density.
0f the following, the MOST common site of fracture is
A. distal tibia
B. supracondylar region of the femur
C. lumbosacral spine
D. proximal humerus
E. distal radius
4. Oral medications are often used as an early treatment for generalized spasticity.
Which of the following works at the level of skeletal muscle to block calcium release
from the sarcoplasmic reticulum?
dantrolene sodium
clonidine
NP??? tizanidine
baclofen
diazepam
5. Coadministration of botulinum toxin (BTX) and one of the following drugs should be
performed with caution as the effect of the toxin may be potentiated
567
A. aminoglycosides
B. cephalosporines
C. penicillins
D. macrolids
E. sulphonamides
7. The baby with birth brachial plexus palsy (BBPP)will start with occupational or
physical therapy at approximately
A. 1 wk of age
B. 2 wk of age
C. 3 wk of age
D. 4 wk of age
E. 5 wk of age
8. Prenatal screening is recommended for all pregnant women to detect neural tube
defect. If a neural tube defect is present, one of the following is often elevated
human chorionic gonadotropin
a-fetoprotein
WPDP? estriol
inhibin
prolactin
568
- Rehabilitation
Medicine
ANSWERS
ZUHAIR ALMUSAWI
569
Hazards
Health
Environmental
-
QUESTIONS
UHAIR ALM USAWI
radiation
1.Inutero isassociated
exposure fromleukemia
riskofdying
withanexcess
before age 10 yr. .
Of the following,the MOSTacceptableriskpercentrs
A. 32%
B. 52%
C. 75%
D. 92%
E. 180%
3. The acute effects of radiation therapy (occurring less than 3 mo after therapy begins)
are usually related to the area of the body being irradiated.
Of the following, the MOST severe acute reactions is
A. pneumonitis
8. dermatitis
C. mucositis
D. esophagitis
E. cerebral edema
5. Childrenexposedto second-hand
tobaccosmokehaveincreasedfrequencyof
A. middle ear effusions
B. bacterial respiratory illnesses
570
c. otitis externa
D. sinusitis
E. tonsilitis
.mpow?skin tumor
lymphoma
carcinoma of lungs
7. Arsine gas is colorless, odorless, nonirritating, and highly toxic. Inhalation causes no
immediate symptoms. After a latent period of 2-24 hr, exposed individuals experience
A. hematuria
B. sensorimotor peripheral neuropathy
C. respiratory failure
D. massive hemolysis
E. proteinuria
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E. 19% of cases
food
areanideal
11.Mushrooms theyare
because
A. low in calories
B. highin calories
C. high in fat
D. low infat
E. lowin protein
isnotrecommended
12.Oneofthefollowing ofAmanita
fortreatment poisoning
A. oral activated charcoal
B. forced diuresis
C. silibinin
D. intravenous penicillin G
E. acetylcysteine
@909? hypotension
vomiting
bronchospasm
14. Pediatricians
are likelyto experienceunique problems in managingchildhood
victims of biologic or chemical attack.
The very rapid onset of neuromuscular
symptomsafter an exposure should lead the
clinician to consider
botulism
nerve agent intoxication
W995? chlorine
phosgene
cyanide
572
D. anthrax
E. sarin
17. In casesin which the delayed onset of respiratory symptoms may be the result of a
terrorist attack, consideration should be given to the empirical administration of an
antibiotic effective against anthrax, plague, and tularemia. A reasonable choice is
A. ciprofloxacin
B. rifampin
C. vancomycin
D. ampicillin
E. clarithromycin
18. Infection is the most common complication of bite injuries, regardless of the
species of biting animal.
The MOST common microorganisms associated with cat bites are
A. Staphylococcus species
B. Streptococcus species
C. Eikenella species
D. Pasteurella species
E. Proteus species
19. The MOST important proposed field treatments for snake bites is
A. tourniquets
B immobilizationof injured body part at the level of the heart
C. ice
D. incision
E. suction
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A. AV is capable of neutralizing only circulating venom
B. it is beneficial to give AV locally at the bite site
C. most AVs are given intravenously
D. skin tests are unreliable
E. intravenous AV should be started slowly
574
EnvironmentalHealthHazar
ANSWERS
HAIRALMUSAWI
VVVVVVV
Down syndrome
Fanconi anemia
Gardner Syndrome
Nijmegen breakage syndrome
Usher syndrome
3.(A). The acute effects of therapy (occurring less than 3 mo after therapy begins) are
usuallyrelated to the area of the body being irradiated (except fatigue, which can begin
during this time period). These acute effects include radiation-caused pneumonitis,
dermatitis, mucusitis and esophagitis, cerebral edema, and swelling of the organ
irradiated.
There may be changes in bowel movement patterns. Of these, one of the most severe
acute reactions is pneumonitis. It can be manifest within 24 hr of irradiation when
there is an exudation of proteinaceous material into the alveoli and intraalveolar
edema. Most often, however, radiation pneumonitis begins 2-6 mo after the beginning
of radiation with a clinical presentation of fever, cough, congestion, and pruritic pain.
4.(C). Primary malignancies with the highest cumulative incidence of a second
neoplasm in the order of frequency are Hodgkin disease (7.6), soft tissuesarcoma (4.0),
cancersof bone (3.3), leukemia (2.1), central nervoussystEm(CNS)cancers(2.1),and
nonHodgkin disease lymphoma (1.9).
S.(A).Children exposed to second-handtobacco smoke have increasedfrequency 0f
lower respiratory illness, more middleear effusions, and more viral reSpiratory
illnessesthan unexposed children.
6(8).ChildrenWhogrowuponfarmshaveelevated
ratesof leukemia; 3"
pesticides
0f platlnganetiologic
SUSPeCtEd role.
7.(o). After a latent period of 2-24 hr, expOsedindividualsexperiencemassive
malaise:
hemolysis, weakness,
headache, nausea,
dyspnea, P33".
abdominal
Vomiting,
hepatomegaly,
(Jai'or, andrenal
hemoglobinuria, failure.
jaundice,
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8.(D). Acuteingestion of inorganicmercury salts(typically secondary to ingestion of a
button battery) can manifest in a few hours as corrosive gastroenteritis, signified by
metallic taste, oropharyngeal burns, nausea, hematemesis, severe abdominal pain,
hematochezia, acute tubular necrosis, cardiovascular collapse, and death.
9.(E). The symptom complex includes generalized pain, paresthesias, and an acral
(hands, feet) rash that may spread to involve the face. The rash typically is red-pink,
papular, pruritic, and painful; it may progress to desquamation and ulceration.
Morbilliform, vesicular, and hemorrhagic variants have been described. Other
important features include anorexia, apathy, photophobia, and hypotonia, especially of
the pectoral and pelvic girdles. Irritability, tremors, diaphoresis, insomnia,
hypertension, and tachycardia may be present. Some cases initially were diagnosed as
pheochromocytoma.
10.(A). It is estimatedthat 99% of lead-poisoned children are identified by screening
procedures rather than through clinical recognition of lead related symptoms.
11.(A). Mushrooms are an ideal food. They are low in calories, fat free, and high in
protein, making them a great source of nutrition.
12.(B). Forced diuresis should be avoided, as this increases renal exposure.
13.(E). Respiratory distress caused by bronchospasm and increased bronchopulmonary
secretions is the most serious complication.
14(8). The very rapid onset of neuromuscular symptoms after an exposure should lead
the clinician to consider nerve agent intoxication. The nerve agents (tabun, sarin,
soman, and VX) are organophosphate analogs of common pesticides that act as potent
inhibitors of the enzyme acetylcholinesterase. They are hazardous via ingestion,
inhalation, or cutaneous absorption.
15.(D). A delayed onset of respiratory symptoms (days after exposure) is characteristic
of several infectious diseases and 1 toxin that might be adapted for sinister purposes by
terrorists. Among the most threatening and problematic of these are anthrax, plague,
tularemia, and ricin, the latter having garnered considerable media attention in recent
years.
16.(E). Pralidoxime (also known as 2-PAM) cleaves the organophosphate moiety from
cholinesterase and regenerates intact enzyme if aging has not occurred. The effect is
most prominent at the neuromuscular junction and leads to improved muscle strength.
lts prompt use (at a dose of 25 mg/kg) as an adjunct to atropine is recommended in all
serious cases.
576
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.
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and suction,haveproVe ineffecti
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deietenous.
. . oftenrecommended byAV
Skintests are and
manufacturers, ou
unreliable
shId
20.(B).
omitted.
be Two
21.3. ' - thatcanbeconfused
im mgestuons bark
Scorpio
The
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"8 and
poisoning
rfjinOphosphate
asgrzint
movements
seen me with
methampheta
inbark Intoycatiom
scorpion are
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may differentiate
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Ingestions these
cEFLYenomations
on Itions.
_ Medicine
Laboratory
QUESTIONS
SAMA AUUMAILY
2. The most commonly used reference range is generally given as the mean of the
reference population 12 standard deviations (SD).
The term "normal distribution" refers to which of the following?
A. gaussian distribution
8. exponential distribution
C. skewed distribution
D. uniform distribution
E. unimodal distribution
578
5. Assumeyou are going to estimate the prevalence of amoebic dysentery in a small
country which harbors a total number of population of 530,000; you find that 57,000 of
the population are infected by the disease. The prevalence of this disease is closest to
A. 5.33%
B. 7.45%
C. 10.75%
D. 20.22%
E. 25.3%
6. Assume you have the results of PCR tests for Mycoplasma pneumoniae; the number
of positive tests is 83 collected from a 100 truly-infected persons.
The sensitivity of this test is
A. 17%
B. 55%
C. 60%
D. 83%
E. 95%
7. Assume you are evaluating the specificity of the indirect fluorescence antibody test
for rapid diagnosis of Campylobacter enteritis. The number of normal persons tested is
150 and the test is positive in 30 persons. The specificity of this test is
20%
40%
PPS? 60%
70%
80%
8. You are assigned to evaluate the outcome of fecal occult blood test as a screening
test for patients with bowel cancer. The total number of patients who underwent
endoscopy is 2030; in those whom the disease is confirmed are 30. The results of fecal
occult blood test are as follows: positive in 20 patients who has a confirmed bowel
cancer (truly positive), and in 180 patients who dont have the disease (falsely positive);
and negative in 10 patients who truly has the disease (falsely negative), and in 1820
patients who are not affected by the disease (truly negative).
All the following statements are true EXCEPT
A. the prevalence of the disease for those population is 1.48%
B. the sensitivity of the test is 67%
C. the specificity of the test is 91%
D. the positive predictive value is 20%
E. the negative predictive value is 99.5%
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9. Rapid HIV antibody testing procedures using a finger stickor venipuncture to obtain
whole blood, plasma, or serum, andtests using oral fluid were approved.
All the following regarding this test are true EXCEPT
A. they are simple and accurate as to render the likelihood of an erroneous result
by the user negligible
B. a positive result does not need confirmation by Western blot analysis or
immunofluorescence assay
C. they allow for implementation of antiretroviral therapy for HIV-infected
women who have not been tested or are unaware of their HIV status
D. they significantly reduce the risk of mother-tochild transmission
I The median turnaround time for obtaining results from blood collection to
patient notification was only 66 minutes
10. Almost all of the diseases detected in neonatal screening programs have a very low
prevalence; the strategy is to use the initial screening test to separate a highly suspect
group of patients from normal infants (i.e., to increase the prevalence) and then to
follow this suspect group aggressively.
Of the following, the disease that has low prevalence rendering it not useful for
neonatal screening testing is
A. phenylketonuria
B. hypothyroidism
C. sickle cell disease
D. cystic fibrosis
E. neuroblastoma
_ Medicine
Laboratory
ANSWERS
ySAMA AUUMAILY
1.(E).Other considerationsfor further defining reference intervals include partitioning
basedon sex and age.
2.(A).All others are non-normal distribution.
3.(A). Accuracy is a measure of the nearness of a test result to the actual value,
whereas precision is a measure of the reproducibility of a result.
MB). Precision is the repeatability of the measurement. The measurement can be
precisebut not accurate, accurate but not precise, both accurate and precise, or not
accurate nor precise.
5.(C).Prevalence refers to the percentage of the infected people from the total number
of population.
6.(D). Sensitivity means the probability that a patient who is disease positive will test
positive. Sensitivity = number positive by testXIOO /total number positive.
7.(E). Specificity (also called the true negative rate) measures the proportion of
negatives that are correctly identified as such (e.g., the percentage of healthy people
who are correctly identified as not having the condition).
Specificity = number negative by testx100 [total number without disease.
8.(D). PV of a positive test result = True-positive resultsx 100 /Total positive results. PV
of a negative test result = True-negative resultsx 100 /Total negative results.
9.(D). A positive rapid HIV test result is then confirmed by Western blot analysis or
immunofluorescenceassay. Rapid whole blood HIV testing is now the standard of care
for women in labor with undocumented HIV status. Rapid HIV testing can also be used
in developing countries. In resource-poor settings, because of the lack of properly
equipped laboratories, skilled technologists, and basic resources, such as electricity and
water.
10.(E). A simple spot test for VMA is not useful in general screening programs because
of the low prevalence of neuroblastoma (3 cases/100,000) and the low sensitivity of
the test (69%). Sickle cell disease, easily detected using liquid chromatography or
isoelectric focusing, can betreated more effectively if it is diagnosed before clinical
signs appear.ln addition, the results of neonatal screening for cystic fibrosis show that
there are clear benefits associated with preclinical diagnosis.
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Thanks for Allah
