Treatment of nephrogenic diabetes insipidus with hydrochlorothiazide and amiloride

Nephrogenic diabetes insipidus (NDI) is characterised by the inability of t he kidney to concentrate urine in response to arginine vasopressin. The con sequences are severe polyuria and polydipsia, often associated with hyperto nic dehydration. Intracerebral calcification, seizures, psychosomatic retar dation, hydronephrosis, and hydroureters are its sequelae. In this study fo ur children with NDI were treated with 3 mg/kg/day hydrochlorothiazide and 0.3 mg/kg/day amiloride orally three times a day for up to five years. whil e undergoing treatment, none of the patients had signs of dehydration or el ectrolyte imbalance, all showed normal body growth, and there was no eviden ce of cerebral calcification or seizures. All but one had normal psychomoto r development and normal sonography of the urinary tract. However, normal f luid balance was not attainable (fluid intake, 3.8-7.7 1/m(2)/day; urine ou tput, 2.2-7.4 1/m(2)/day). The treatment was well tolerated and no side eff ects could be detected. Prolonged treatment with hydrochlorothiazide/amilor ide appears to be more effective and better tolerated than just hydrochloro thiazide Its efficacy appears to be similar to that of hydrochlorothiazide/ indomethacin but without their severe side effects.