 All patients met at least three standard criteria for the disease including hypercalciuria, low molecular weight proteinuria and one of the following disorders: nephrocalcinosis, nephrolithiasis, renal failure, aminoaciduria, glucosuria, renal phosphate wasting, or familial history of Dents disease. The disease was confirmed in all patients by direct sequencing of the CLCN5 gene according to Lloyd et al. 25 Patients presenting hyponatremia 135 mM, hypokalaemia 3.3 mM, severe fanconi syndrome, or chronic renal failure GFR estimated with the Schwartz formula 30 mL.min-1.1.73m-2 were excluded from the study. 