Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologie · 2019
[Urinary disorders of Wolfram syndrome. Clinical and urodynamic analysis from 6 observations].
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Leroux C, Grasland M, Turmel N, Le Breton F, Chesnel C, Hentzen C, Amarenco G
Аннотация
UNLABELLED: Wolfram syndrome is a neurodegenerative disorder characterized by childhood onset diabetes mellitus, optic nerve atrophy, diabetes insipidus, hearing impairment, brainstem alteration and commonly bladder and bowel dysfunction.
OBJECTIVE: We present here, 6 new cases of urinary dysfunction in this rare disease.
RESULTS: All patients had urinary retention with overactive bladder. The urodynamic assessment found overactive detrusor in 3 cases. Five out of six patients performed self-catheterization and were treated with anticholinergics or intradetrusor injection of botulinum toxin. The follow-up at 5 years found an alteration of the upper urinary tracts and a renal failure 3/6.
CONCLUSION: Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients.
LEVEL OF EVIDENCE: 4.
OBJECTIVE: We present here, 6 new cases of urinary dysfunction in this rare disease.
RESULTS: All patients had urinary retention with overactive bladder. The urodynamic assessment found overactive detrusor in 3 cases. Five out of six patients performed self-catheterization and were treated with anticholinergics or intradetrusor injection of botulinum toxin. The follow-up at 5 years found an alteration of the upper urinary tracts and a renal failure 3/6.
CONCLUSION: Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients.
LEVEL OF EVIDENCE: 4.