title: [Primary immunodeficiency disorders in 51 cases].
abstract: BACKGROUND
  Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections.
  
  
  OBJECTIVE
  To determine the frequency of PID in south of Tunisia to collect information on clinical experience with these disorders.
  
  
  METHODS
  Over a period of 16 years (1995 - 2010), primary immunodeficiency was confirmed in 51 patients (31 boys and 20 girls). The immunological investigation included a study of specific and/or non specific humoral and cellular immunity.
  
  
  RESULTS
  These 51 patients belonged to 47 families among which 37 were consanguine (80%). The immunological investigations revealed a cellular or combined immunodeficiency in 21 cases, with a majority of ataxia-telangiectasia syndromes (11 cases), HLA class II deficiency (9 cases). A predominant antibody defect was found in 3 patients and a chronic granulomatous disease in seven cases. Deaths occurred So far in 19 patients (37 %).
  
  
  CONCLUSIONS
  PID are relatively frequent in Tunisia, probably because of the high rate of consanguinity among the general population. The distribution of the different groups of primary immunodeficiencies is characterized by high frequency of ataxia - telangiectasia and HLA class II deficiency.
authors: Service de pédiatrie du CHU Hédi Chaker de Sfax.
authors: Sfaihi
authors: S
authors: Lamia
authors: Hajer
authors: H
authors: Aloulou
authors: Thouraya
authors: T
authors: Kamoun
authors: Imen
authors: I
authors: Chabchoub
authors: Imen
authors: I
authors: Ben Moustapha
authors: Rhidha
authors: R
authors: Barbouch
authors: Hachicha
authors: H
authors: Mongia
mesh_terms: D002648:Child; D002675:Child, Preschool; D003663:Decision Trees; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000453:epidemiology*; D007223:Infant; D008297:Male