Dose-Dependent Effect of Thiazide in Dent's Disease Hypercalciuria (DESY)
The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government.
Read our disclaimer for details.
Intrarenal calcifications (nephrocalcinosis) is present in Dent's disease and likely contribute to progression toward renal failure. In order to prevent this complication it is usually proposed to treat affected patients during childhood with high doses of thiazides.
to investigate the dose-dependent benefit/risk ratio of low (6.25 mg/day), intermediate (12.5 mg/day) and high (25 mg/day) doses of hydrochlorothiazide [ Time Frame: at the end of the baseline initial and final washout periods (one month each) and at the end of each two-months duration treatment period, ]
Secondary Outcome Measures
clinical events (cramps, symptomatic dehydration, acute weight loss) [ Time Frame: at least two times overall the study, at J15 and at the end of each period. ]
Biological : acute renal failure (estimated GFR), hypokalemia, hyperkalemia, hyponatremia, biological signs of extra cellular dehydration (protides, hematocrit, plasma active renin, plasma aldosterone) [ Time Frame: at least two times overall the study, at J15 and at the end of each period. ]
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.
Ages Eligible for Study:
3 Years to 18 Years (Child, Adult)
Sexes Eligible for Study:
Accepts Healthy Volunteers:
Children > 3 yrs old who met at least three standard criteria for Dent's 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 Dent's disease.
Dent's Disease confirmed by direct sequencing of the CLCN5 gene according to Lloyd et al.
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.