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[Urologic manifestations in Wolfram's syndrome]

J Monllor Gisbert1, F Taño Pino, P Rodríguez Arteaga

  • 1Servicio de Urología, Complejo Hospitalario Ntra, Sra. de Candelaria, Santa Cruz de Tenerife.

Actas Urologicas Espanolas
|May 1, 1996
PubMed
Summary

Wolfram syndrome, a rare genetic disorder, can present with significant urological issues like ureterohydronephrosis and neurogenic bladder. These conditions improved with antibiotics and intermittent catheterism.

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Area of Science:

  • Genetics
  • Urology
  • Endocrinology

Background:

  • Wolfram syndrome is a rare genetic disorder characterized by the association of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness.
  • Urological complications are not commonly highlighted in Wolfram syndrome literature.

Observation:

  • This paper presents two case reports of patients with Wolfram syndrome experiencing significant urological symptoms.
  • The observed urological conditions included ureterohydronephrosis and neurogenic bladder.

Findings:

  • Radiological and urodynamic assessments were performed to evaluate the urological conditions.
  • The study comments on the probable origins and natural evolution of these urological manifestations.

Implications:

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  • The urological symptoms in these Wolfram syndrome cases showed a favorable response to treatment.
  • Treatment involved antibiotics and intermittent vesical catheterism, suggesting potential management strategies for urological complications in Wolfram syndrome.