Progressive hydronephrosis, hydroureter, and dilation of the bladder in siblings with congenital nephrogenic diabetes insipidus

Wolfram's (DIDMOAD) Syndrome and its Urological Manifestation

“…Diabetes insipidus has been one of the main features of Wolfram's syndrome. Although Cremers et al (1977) reported an incidence of only 32%, a detailed study of vasopressin secretion in Wolfram's syndrome reported diabetes insipidus in all 7 patients and concluded that it was present in most cases and was mainly hypothalamic in origin (Thompson et al, 1989). Both of our patients had diabetes insipidus that was also hypothalamic in origin as it responded adequately to dDAVP.…”

Hereditary Nephrogenic Diabetes insipidus and Bilateral Nonobstructive Hydronephrosis

“…Evidence of obstruction was found in only 3 of these patients in the form of bladder neck hypertrophy, which may itself be a conse quence of polyuria [10], This strong association between hereditary nephrogenic diabetes insipidus and nonobstruc tive hydronephrosis suggests a cause-and-effect relation ship: otherwise, it would have to be postulated that patients with hereditary nephrogenic diabetes insipidus inherit two defects: an inability to concentrate the urine and a tendency for dilatation of the urinary tract. We think that the obvious cause of the dilatation of the urinary system is the high urinary output; when the rate of How exceeds the emptying capacity of a conduit, a functional obstruction will occur and, if the tube is distensible, dilatation will follow as the tube cannot empty all the fluid it receives [9]. 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].…”

“…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.…”

Hypertensive urgency in nephrogenic diabetes insipidus with concomitant Hinman syndrome

“…Nonetheless, the primary management of NDI is to reduce polyuria and decrease the feeling of thirst. Optimal pharmacotherapy facilitates a reduction in urinary water losses,9 thereby preventing urinary tract dilatation 10. This can also optimise growth with a decreased incidence of mental retardation 11.…”