Three cases of didmoad or Wolfram's syndrome: urological aspects.
We report on 3 patients with the rare syndrome of diabetes insipidus, diabetes mellitus, optic atrophy, neurosensory deafness, atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome). All 3 patients had diabetes mellitus, optic atrophy, deafness and dilatation of the urinary tract. In 2 patients there was diabetes insipidus. The possibility of anatomical outlet obstruction or a neurogenic bladder was eliminated radiologically and urodynamically, and dilatation of the urinary tract was considered to be either a consequence of high diuresis associated with diabetes insipidus or a degenerative process affecting the central and peripheral nervous system, which can explain all of the manifestations of the syndrome except diabetes mellitus. A significant improvement in bilateral urinary tract distention was achieved by bladder drainage in the first 2 cases, while desmopressin therapy dramatically decreased the daily urinary output.
Thanos
A
A
Department of Urology, General Hospital of Athens, Greece.
Farmakis
A
A
Sami
Z
Z
Davillas
E
E
Davillas
N
N
D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D014570:Urologic Diseases / Q000209:etiology*; D014929:Wolfram Syndrome / Q000150:complications* / Q000235:genetics