title: Incidence of mucopolysaccharidoses in Tunisia.
abstract: BACKGROUND
  The mucopolysaccharidoses (MPS) are a devastating heterogenous group of lysosomal storage disorders.
  
  
  OBJECTIVE
  To evaluate the epidemiological profile of MPS in Tunisia.
  
  
  METHODS
  we conducted a retrospective epidemiological survey covering the period 1970-2005. Multiple sources were used to identify affected patients.
  
  
  RESULTS
  Ninety six confirmed MPS cases were collected from 132 suspected cases found in the surveyed data. Of the ninety six confirmed cases, 20% were from multiplex families. Consanguinity was found in 83% of the families. The crude rate for all types of mucopolysaccharidoses was 2.3 cases in 100,000 live births. The prevalence of MPS type I, III and IV, those most frequently occurring in the collected data, were estimated at 0.63, 0.7 and 0.45 per 100,000 live births, respectively. The cumulative incidence of MPS type VI (0.3 per 105 live births) was higher than reported in European countries; but, it is likely that...
  
  
  CONCLUSIONS
  The reported frequency of all types of MPS in Tunisia is underestimated.
authors.affiliation: Paediatric Department, La Rabta Hospital, Tunis
authors.forename: Hadhami
authors.initials: H
authors.lastname: Ben Turkia
authors.forename: Néji
authors.initials: N
authors.lastname: Tebib
authors.forename: Hatem
authors.initials: H
authors.lastname: Azzouz
authors.forename: Mohamed S
authors.initials: MS
authors.lastname: Abdelmoula
authors.forename: Amal
authors.initials: A
authors.lastname: Ben Chehida
authors.forename: Jalel
authors.initials: J
authors.lastname: Chemli
authors.forename: Kamel
authors.initials: K
authors.lastname: Monastiri
authors.forename: Malak
authors.initials: M
authors.lastname: Chaabouni
authors.forename: Haifa
authors.initials: H
authors.lastname: Sanhagi
authors.forename: Béchir
authors.initials: B
authors.lastname: Zouari
authors.forename: Naziha
authors.initials: N
authors.lastname: Kaabachi
authors.forename: Marie F
authors.initials: MF
authors.lastname: Ben Dridi
mesh_terms: D003241:Consanguinity; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009083:Mucopolysaccharidoses / Q000453:epidemiology*; D012189:Retrospective Studies; D014416:Tunisia / Q000453:epidemiology