[Wolfram syndrome. Report of 3 cases].
Wolfram's syndrome is an autosomal recessive neurodegenerative disease. The major manifestations consist of diabetes mellitus and bilateral optic atrophy and are sufficient to establish the diagnosis. Urinary manifestations constitute an integral part of the syndrome, as they are present in up to 62% of cases and represent one of the major causes of morbidity. Three cases allow emphasize the difficulty of treatment, which is essentially symptomatic, and largely guided by the results of the various complementary investigations, especially urodynamic assessment. Although the causes of this syndrome are multifactorial and complex, it appears that the urinary lesions are at least temporarily improved by correction of excessive bladder pressure.
Benchekroun
A
A
Clinique Urologique A, CHU Avicenne, Rabat, Maroc.
Nouini
Y
Y
Iken
A
A
Iraqui
M
M
el Alj
H A
HA
Jdoud
C O
CO
D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D014929:Wolfram Syndrome / Q000175:diagnosis / Q000628:therapy*