Nephrogenic Diabetes Insipidus Accompanied by Massive Dilatation of the Kidneys, Ureters and Bladder

DIDMOAD syndrome with megacystis and megaureter

“…Page et al (1976) have suggested that the high urine flow rate caused by diabetes insipidus leads to progressive passive dilatation of the urinary tract and that this together with inattention to periodic bladder emptying impairs detrusor action producing a functional obstruction. This explanation is consistent with the observation that megaureter and megacystis do occur in both hereditary nephrogenic diabetes insipidus (Carter & Goodman, 1963;ten Bensel & Peters, 1970) and hereditary anti-diuretic hormone sensitive diabetes insipidus (Pender & Fraser, 1953;Wheeler & Adelson, 1964;Boyd et al, 1980). Urinary tract dilatation does not develop in adult onset diabetes insipidus (Fink, 1928;Page et al, 1976) but this might be because the high flow rate operates over a shorter period or because the childhood tract is more susceptible to dilatation.…”

Hereditary Nephrogenic Diabetes insipidus and Bilateral Nonobstructive Hydronephrosis

“…I n the absence of dehydration, renal function is said to be normal [46]. However, our 2 patients had mild compromise of glomerular filtration rate and hypertension, which have been described in several of these patients in previous reports [4,7,8,11,16,42], but the exact incidence of decline of renal function in this condition remains unknown.…”

“…Further support for the theory that polyuria causes dilatation of the urinary tract comes from the observation that bilateral hydronephrosis has also been described in patients with pituitary diabetes insipidus [39^43], in patients with primary polydypsia and in experimental models of nephrogenic diabetes insipidus in mice [44] and hypothalamic pituitary diabetes insipidus in rats [45]. The dilatation is less likely to occur in the acquired form of pituitary diabetes insipidus because the disease is usually of short duration; instead patients with hereditary nephrogenic diabetes insipidus present with massive polyuria for prolonged periods of time and from an early stage of development, when the GU tract may be more susceptible to undergo dilatation [7], Ten Bensel [9] has postulated that the patient forced with massive urine output performs repeated voluntary retention of urine for social reasons which may lead to secondary hypertrophy of the sphincter.…”

Suppressed urinary excretion of aquaporin-2 in an infant with primary polydipsia

“…MRI of the urinary tract showed bilateral hydronephrosis and marked dilatation of the bladder due to severe overhydration, which is also observed in patients with diabetes insipidus [11][12][13].…”