Incidence of mucopolysaccharidoses in Tunisia

Hadhami Ben Turkia1, Néji Tebib, Hatem Azzouz

  • 1Paediatric Department, La Rabta Hospital, Tunis

La Tunisie Medicale
|March 10, 2010
PubMed
Abstract

Insights

The study found the frequency of mucopolysaccharidoses (MPS) in Tunisia is likely underestimated, with a crude rate of 2.3 cases per 100,000 live births. Consanguinity was high in affected families.

Area of Science:

  • Medical Genetics
  • Epidemiology
  • Lysosomal Storage Disorders

Background:

  • Mucopolysaccharidoses (MPS) are a group of rare, inherited metabolic diseases.
  • These lysosomal storage disorders lead to severe health complications.

Purpose of the Study:

  • To determine the epidemiological characteristics of MPS in Tunisia.
  • To establish the prevalence and incidence of different MPS types in the region.

Main Methods:

  • A retrospective epidemiological survey was conducted from 1970 to 2005.
  • Data was collected from multiple sources to identify patients with suspected or confirmed MPS.

Main Results:

  • Ninety-six confirmed MPS cases were identified out of 132 suspected cases.
  • The crude incidence rate for all MPS types was 2.3 per 100,000 live births.
  • Prevalence rates for MPS I, III, and IV were 0.63, 0.7, and 0.45 per 100,000 live births, respectively. MPS VI incidence was higher than in European populations.

Conclusions:

  • The actual frequency of MPS in Tunisia is likely higher than reported.
  • High consanguinity rates (83%) in affected families suggest a genetic component.
  • Further research is needed to fully understand the epidemiology of MPS in Tunisia.

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