Idiopathic hypercalciuria (IH) associated with nephrocalcinosis was fo
und in three of six siblings. After the three affected children were m
aintained on a low-calcium diet, they demonstrated increasing hypercal
ciuria, parathyroid hormone, and vitamin D-3 levels. An oral calcium l
oading test was not necessary to diagnose renal IH. During treatment w
ith hydrochlorothiazide, the calcium excretion was normalized. These p
atients are remarkable because nephrocalcinosis is generally regarded
as a rare complication of renal M. Moreover, the fact that three of si
x siblings are affected raises the question of whether the renal form
of IH is genetically distinct from other forms of IH.