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Helen K Burgess, Satishkumar A Jayawardene, Nestor Velasco, Dent's disease: can we slow its progression?, Nephrology Dialysis Transplantation, Volume 16, Issue 7, July 2001, Pages 1512–1513, https://doi.org/10.1093/ndt/16.7.1512
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Sir,
Dent's disease is a syndrome of low molecular weight proteinuria, hypercalciuria, aminoaciduria and hypophosphataemia. It was first described by Dent and Friedman in 1964 in two unrelated boys with rickets [1]. The condition is familial, affecting both males and females in equal numbers, but males are more severely affected than females. In males, it tends to present in childhood or early adult life with symptoms of renal calculi, rickets or even with renal failure. It is still unclear as to exactly why and what proportion of sufferers progress to end‐stage renal failure (ESRF). The nephrolithiasis associated with Dent's disease usually takes the form of nephrocalcinosis [2]. It is thought that the decline in renal function may be partially due to the infection and obstruction associated with nephrocalcinosis. This however, cannot be the sole reason, as some patients have reached ESRF with no evidence of it [2]. In renal transplant recipients, there is no recurrence of stone disease within the renal allograft, suggesting an intrinsic renal problem [3]. In the largest study of patients with Dent's disease, nine out of 15 men and one out of 10 women reached ESRF (at a mean age of 47 years) [2]. At present there is no strategy for the treatment of patients other than supportive measures.
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