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Urinary Tract Involvement in Wolfram Syndrome: A Narrative Review.
Alberto La Valle1,2, Gianluca Piccolo1,2, Mohamad Maghnie1,2,3
1Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health University of Genoa, 16147 Genoa, Italy.
International Journal of Environmental Research and Public Health
|November 27, 2021
Summary
Wolfram Syndrome (WS) significantly impacts the urinary tract in 90% of patients, often leading to life-threatening kidney failure. This review highlights crucial urological aspects in WS management.
Area of Science:
- Nephrology
- Urology
- Genetics
Background:
- Wolfram Syndrome (WS) is a rare, autosomal recessive neurodegenerative disorder.
- Key features include juvenile diabetes mellitus, optic atrophy, and progressive neurological dysfunction.
- Urinary tract dysfunctions (UTD), including neurogenic bladder and end-stage renal disease (ESRD), are severe complications.
Purpose of the Study:
- To provide a comprehensive overview of urological impairment in Wolfram Syndrome.
- To synthesize current literature on urinary tract dysfunction in WS patients.
- To emphasize the significance of urological complications in WS prognosis.
Main Methods:
- A narrative review methodology was employed.
- A comprehensive literature search was conducted on PubMed.
- Keywords included Wolfram Syndrome, chronic renal failure, bladder dysfunction, urological aspects, and urinary tract dysfunction.
Main Results:
- Urinary tract involvement affects approximately 90% of WS patients, often manifesting by age 20.
- End-stage renal disease is a frequent and life-threatening complication, contributing to mortality.
- Urodynamic abnormalities and neurogenic bladder are significant concerns in WS management.
Conclusions:
- Urological complications are a major concern in Wolfram Syndrome, significantly impacting patient morbidity and mortality.
- Early recognition and management of UTD are critical for improving outcomes in WS patients.
- Further research is needed to elucidate the mechanisms and optimize treatments for urinary tract dysfunction in WS.
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