Two siblings with nephrogenic diabetes insipidus are reported.
One had unilateral obstructive uropathy due to an ectopic ureterocele; this was associated with a sodium-wasting state which led to transient sodium depletion postoperatively.
His older affected sister had massive.dilatation of the entire urinary tract, but no evidence of organic obstruction or vesicoureteric reflux. Upper tract dilatation is believed to result from functional obstruction at the ureterovesical junction, due to the greatly increased flow of urine and marked distension of the bladder. The reservoir function of the bladder is overloaded by the polyuria and by voluntary retention, leading to marked bladder enlargement.