先天性腎性尿崩症によるPolyuric Megalocystisの2例

Abstract

We have diagnosed and treated two siblings with megacystis and hydronephrosis secondary to polyuria, resulting from congenital nephrogenic diabetes insipidus. This report mainly refers to the voiding dysfunction of the urinary bladder in the patients. The patients had neither neurological disorder, anatomical outlet obstruction nor vesico-ureteral reflux. Urodynamically, both of them showed a normal urethral pressure profile, but the cystometric findings were different; an uninhibited bladder in the elder brother, a high compliance bladder in the younger brother. The difference between these cystometic findings is discussed on the basis of functional changes of the overdistended detrusor.