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Wolfram's (DIDMOAD) syndrome and its urological manifestation
S Aboseif1, M Gasparini, R Schmidt
1Department of Urology, University of California School of Medicine, San Francisco.
British Journal of Urology
|July 1, 1993
Summary
This study details two cases of DIDMOAD syndrome, focusing on the urological issues and management. Both patients presented with neurogenic bladder, leading to severe urinary tract dilation and requiring tailored interventions.
Area of Science:
- Urology
- Endocrinology
- Genetics
Background:
- DIDMOAD syndrome, also known as Wolfram syndrome, is a rare autosomal recessive disorder.
- It is characterized by the combination of diabetes insipidus, diabetes mellitus, optic atrophy, and neural deafness.
- Urological complications are significant but often under-recognized aspects of this syndrome.
Observation:
- Two patients with DIDMOAD syndrome were evaluated for urological manifestations.
- Both exhibited a hyper-reflexive neurogenic bladder with sphincteric dyssynergia.
- This resulted in severe urinary tract dilation, necessitating comprehensive assessment.
Findings:
- Urodynamic evaluation revealed characteristic findings consistent with detrusor-sphincter dyssynergia.
- Conservative management with clean intermittent catheterization and anticholinergic medication was successful in one patient.
- The second patient required surgical correction after previous failed attempts, highlighting the complexity of management.
Implications:
- Effective management of neurogenic bladder in DIDMOAD syndrome is crucial to prevent upper tract deterioration.
- Urological assessment and urodynamics are essential for accurate diagnosis and treatment planning.
- A multidisciplinary approach is vital for optimizing outcomes in patients with DIDMOAD syndrome.