title: [Prevalence of hemoglobin abnormalities in Kebili (Tunisian South)].
abstract: BACKGROUND
  Hemoglobin abnormalities constitute a public health problem in many countries in the world. In Tunisia, these disorders were thought to affect only the North-western population. However, the existence of hemoglobinosis concentration in Kebily in south Tunisia has been suggested by previous work. In order to estimate their frequencies, we performed a screening of hemoglobin abnormalities in the North-Kebili region, to establish a prevention program of the homozygous forms.
  
  
  METHODS
  This screening concerned all 1st and 2nd grade primary school pupils in North Kebily. After a questionnaire, a blood sample was drawn from every child. Hemogram, sickling test, and hemoglobin electrophoresis at alkaline pH were performed for all children. Hemoglobin electrophoresis at acid pH and a specific hemoglobin A2 titration were performed for some children.
  
  
  RESULTS
  The study concerned 1,400 children, aged between 5 and 12 years, the mean age was 7 years and 7 months +/- 10 months. Consanguinity rate and coefficient were respectively 44% and 2249 x 10(5). Endogamy was very high. The global rate of hemoglobin abnormalities was 9.4%. Drepanocytosis with a rate of 4.9% was the most frequent, followed by beta thalassemia (3.1%) and C hemoglobinosis (1.6%). These abnormalities were unequally distributed; very frequent in some localities, they were quite absent in others.
  
  
  CONCLUSIONS
  This study revealed a hemoglobinosis concentration in Tunisia, which can be classified second after that of Beja in North-western Tunisia. The heterogeneous distribution of the hemoglobin abnormalities in North-Kebili region and the high consanguinity and endogamy rates constitute factors that promote homozygous and double heterozygous forms to arise and justify the elaboration of a preventive strategy.
authors.affiliation: Service d'hématologie-C.H.U. Hédi Chaker, Sfax, Tunisie.
authors.forename: S
authors.initials: S
authors.lastname: Mseddi
authors.forename: J
authors.initials: J
authors.lastname: Gargouri
authors.forename: Z
authors.initials: Z
authors.lastname: Labiadh
authors.forename: M
authors.initials: M
authors.lastname: Kassis
authors.forename: M
authors.initials: M
authors.lastname: Elloumi
authors.forename: L
authors.initials: L
authors.lastname: Ghali
authors.forename: J
authors.initials: J
authors.lastname: Dammak
authors.forename: M
authors.initials: M
authors.lastname: Harrabi
authors.forename: T
authors.initials: T
authors.lastname: Souissi
authors.forename: M
authors.initials: M
authors.lastname: Frikha
mesh_terms: D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D003430:Cross-Sectional Studies; D006445:Hemoglobin C Disease / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D006453:Hemoglobinopathies / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008957:Models, Genetic; D014416:Tunisia / Q000453:epidemiology; D017086:beta-Thalassemia / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics