title: First report on the Moroccan registry of primary immunodeficiencies: 15 years of experience (1998-2012).
abstract: OBJECTIVE
  Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to infections. We provide the first comprehensive report on PIDs in Morocco, the epidemiological, clinical, etiological and outcome features which have never before been described.
  
  
  METHODS
  A national registry was established in 2008, grouping together data for PID patients diagnosed since 1998.
  
  
  RESULTS
  In total, 421 patients were diagnosed between 1998 and 2012. Parental consanguinity was common (recorded for 43.2 % of patients) and the median time to diagnosis was 2.0 years. Overall, 27.4 % of patients were considered to have well defined syndromes with immunodeficiency (48 cases of hyper-IgE syndrome and 40 of ataxia-telangiectasia); 22.7 % had predominantly antibody deficiencies (29 cases of agammaglobulinemia and 24 of CVID); 20.6 % had combined immunodeficiencies (37 cases of SCID and 26 of MHC II deficiencies) and 17.5 % had phagocyte disorders (14 cases of SCN and 10 of CGD). The principal clinical signs were lower respiratory tract infections (60.8 %), skin infections (33.5 %) and candidiasis (26.1 %). Mortality reached 28.8 %, and only ten patients underwent bone marrow transplantation. We analyzed the impact on mortality of residence, family history, parental consanguinity, date of diagnosis and time to diagnosis, but only date of diagnosis had a significant effect.
  
  
  CONCLUSIONS
  The observed prevalence of PID was 0.81/100,000 inhabitants, suggesting considerable underdiagnosis and a need to increase awareness of these conditions in Morocco. The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of SCID, MHC II deficiencies, hyper-IgE syndrome and autosomal recessive agammaglobulinemia. However, we have now organized a national network, which should improve diagnosis rates in remote regions.
authors.affiliation: Clinical Immunology Unit, Department of Pediatric Infectious Diseases, Averroes University Hospital, King Hassan II University-Aïn Chok, Casablanca, Morocco, profbousfiha@gmail.com.
authors.forename: A A
authors.initials: AA
authors.lastname: Bousfiha
authors.forename: L
authors.initials: L
authors.lastname: Jeddane
authors.forename: N
authors.initials: N
authors.lastname: El Hafidi
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authors.lastname: Benajiba
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authors.lastname: Rada
authors.forename: J
authors.initials: J
authors.lastname: El Bakkouri
authors.forename: A
authors.initials: A
authors.lastname: Kili
authors.forename: S
authors.initials: S
authors.lastname: Benmiloud
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authors.initials: I
authors.lastname: Benhsaien
authors.forename: I
authors.initials: I
authors.lastname: Faiz
authors.forename: O
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authors.lastname: Maataoui
authors.forename: Z
authors.initials: Z
authors.lastname: Aadam
authors.forename: A
authors.initials: A
authors.lastname: Aglaguel
authors.forename: L Ait
authors.initials: LA
authors.lastname: Baba
authors.forename: Z
authors.initials: Z
authors.lastname: Jouhadi
authors.forename: R
authors.initials: R
authors.lastname: Abilkassem
authors.forename: M
authors.initials: M
authors.lastname: Bouskraoui
authors.forename: M
authors.initials: M
authors.lastname: Hida
authors.forename: J
authors.initials: J
authors.lastname: Najib
authors.forename: H Salih
authors.initials: HS
authors.lastname: Alj
authors.forename: F
authors.initials: F
authors.lastname: Ailal
mesh_terms: D000293:Adolescent; D000328:Adult; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009018:Morocco / Q000453:epidemiology; D015995:Prevalence; D012042:Registries
chemical_list: D016756:Immunoglobulins, Intravenous