First Report on the Moroccan Registry of Primary I

Authors: A A Bousfiha L Jeddane N El Hafidi N Benajiba N Rada J El Bakkouri A Kili S Benmiloud I Benhsaien I Faiz O Maataoui Z Aadam A Aglaguel L Ait Baba Z Jouhadi R Abilkassem M Bouskraoui M Hida J Najib H Salih Alj F Ailal For the Moroccan Society for Primary Immunodeficiencies MSPID

Publish Date: 2014/03/12

Volume: 34, Issue: 4, Pages: 459-468

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Abstract

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 describedIn total 421 patients were diagnosed between 1998 and 2012 Parental consanguinity was common recorded for 432  of patients and the median time to diagnosis was 20 years Overall 274  of patients were considered to have well defined syndromes with immunodeficiency 48 cases of hyperIgE syndrome and 40 of ataxiatelangiectasia 227  had predominantly antibody deficiencies 29 cases of agammaglobulinemia and 24 of CVID 206  had combined immunodeficiencies 37 cases of SCID and 26 of MHC II deficiencies and 175  had phagocyte disorders 14 cases of SCN and 10 of CGD The principal clinical signs were lower respiratory tract infections 608  skin infections 335  and candidiasis 261  Mortality reached 288  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 effectThe observed prevalence of PID was 081/100000 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 hyperIgE syndrome and autosomal recessive agammaglobulinemia However we have now organized a national network which should improve diagnosis rates in remote regions

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