INCIDENCE OF THE MUCOPOLYSACCHARIDOSES IN TUNISIA

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Published Dec 8, 2009
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Vol. 87 No. 11 (2009): Issue Nov 2009

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Hadhami Ben Turkia
Service de Pédiatrie hôpital La Rabta Tunis
Néji Tebib
Hatem Azzouz
Mohamed S. Abdelmoula
Service de Pédiatrie hôpital La Rabta Tunis
Amal Ben Chehida
Service de Pédiatrie hôpital La Rabta Tunis
Jalel Chemli
Kamel Monastiri
Malak Chaabouni
Haifa Sanhagi
Béchir Zouari
Naziha Kaabachi
Marie F. Ben Dridi
Service de Pédiatrie hôpital La Rabta Tunis

Abstract

The mucopolysaccharidoses (MPS) are a devastating heterogenous group of lysosomal storage disorders. To date no comprehensive study has been performed on the prevalence of lysosomal storage disorders in the Tunisian population. In order to create an epidemiological profile of MPS in Tunisia, we conducted a retrospective epidemiological survey covering the period 1970-2005. Multiple sources were used to identify affected patients. 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 the reported frequency of all types of MPS in Tunisia is underestimated.

Keywords:

Mucopolysaccharidoses, Epidemiology, Tunisia

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