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The prevalence of Wolfram syndrome in a paediatric population with diabetes

Agnieszka Zmysłowska, Maciej Borowiec, Wojciech Fendler

  • 1Wojciech.Mlynarski@umed.lodz.pl.

Endokrynologia Polska
|September 5, 2014
PubMed

Insights

Wolfram syndrome (WFS) is a rare genetic disorder affecting children with diabetes. In Poland, WFS affects 0.12% of diabetic children, making it significantly less common than other monogenic diabetes forms.

Area of Science:

  • Endocrinology
  • Genetics
  • Pediatrics

Background:

  • Wolfram syndrome (WFS) is the most common syndromic form of monogenic diabetes.
  • WFS is characterized by diabetes mellitus and optic atrophy, often with other associated disorders.

Purpose of the Study:

  • To determine the prevalence of Wolfram syndrome in the Polish pediatric diabetic population.
  • To compare the frequency of WFS with other monogenic diabetes types in children.

Main Methods:

  • Retrospective analysis of epidemiological data from Polish diabetic children aged 0-18 years (2005-2011).
  • Data linkage between the EURO-WABBPoland Project and the PolPeDiab Registry for confirmed genetic diagnoses.
  • Calculation of prevalence based on diagnosed cases within the study cohort.

Main Results:

  • Genetic diagnosis of Wolfram syndrome was confirmed in 13 pediatric patients in Poland.
  • The overall prevalence of Wolfram syndrome among Polish children with diabetes was estimated at 0.12% (95% CI: 0.04-0.34%).
  • Three WFS cases were identified within the specific regions with complete pediatric diabetes data.

Conclusions:

  • Wolfram syndrome is significantly less frequent than other monogenic diabetes forms (MODY, neonatal diabetes) in Polish children.
  • WFS is estimated to be 26 to 35 times less common than MODY and neonatal diabetes in this population.
Abstract

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