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[Bladder-sphincter disorders associated with Wolfram syndrome].

C Ribière¹, F A Kaboré, A Chaussenot

¹Service de médecine physique et réadaptation, hôpital Archet 1, CHU de Nice, 151, route de Saint-Antoine, 06200 Nice, France.

Progres En Urologie : Journal De L'Association Francaise D'Urologie Et De La Societe Francaise D'Urologie

|June 4, 2013

Summary

Functional urinary symptoms are common in Wolfram syndrome (SW), significantly impacting patients' quality of life. Early urological assessment and consistent follow-up are crucial for managing these conditions and preventing complications.

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Area of Science:

Endocrinology and Metabolism
Urology
Genetics

Context:

Wolfram syndrome (SW) is a rare genetic disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and hearing loss.
Urinary tract complications are frequently observed in SW patients, but their functional impact is not well-characterized.

Purpose:

To identify and describe functional urinary symptoms and uro-nephrological complications in Wolfram syndrome patients.
To assess the impact of these symptoms on the quality of life in this cohort.

Summary:

A cohort study involving 22 Wolfram syndrome patients evaluated urinary symptoms and quality of life using the Urinary Symptom Profile (USP) and ICIQ-FLUTS questionnaires.
Seventy-three percent of patients with spontaneous micturition reported urinary symptoms, with 67% experiencing severe or moderate symptom scores.
Functional urinary dysfunctions were prevalent, affecting over half the patients and impacting their quality of life.

Impact:

Functional urinary dysfunctions are a significant concern in Wolfram syndrome, affecting patient quality of life.
Early diagnosis and regular urological follow-up are recommended to improve outcomes and prevent severe urinary complications in SW patients.