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Primary Immunodeficiencies in Africa: a preliminary idea Bousfiha AA, Esser M, Ailal F, Jeddane L, Boukari R, El Marsafi A, Eley B, Barbouche R, Bejaoui.

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Presentation on theme: "Primary Immunodeficiencies in Africa: a preliminary idea Bousfiha AA, Esser M, Ailal F, Jeddane L, Boukari R, El Marsafi A, Eley B, Barbouche R, Bejaoui."— Presentation transcript:

1 Primary Immunodeficiencies in Africa: a preliminary idea Bousfiha AA, Esser M, Ailal F, Jeddane L, Boukari R, El Marsafi A, Eley B, Barbouche R, Bejaoui M From African Society for ImmunoDeficiencies, website: www.asid.mawww.asid.ma There is renewed interest in Africa for Primary ImmunoDeficiencies, especially since the creation of the African Society for ImmunoDeficiencies (ASID; Casablanca, 2008). So national teams have doubled their efforts and an extraordinary collaboration has been established between the countries where care and research have made some progress. Sensitization sessions and training schools were held in several countries, including Mauritania and South-Africa. The second ASID congress is expected in March 2011 in Tunisia. This work’s purpose is to estimate the number of PIDs in countries who have series, including publications from the works presented during the first ASID congress in 2008. Population (2009)PIDs. *PID patients / year. ** Register of current patients. World6 829 360 438254 052703 424? Africa1 009 892 84437 568104 0191049 Europe732 758 54627 25875 47410 279 Table 1- Number of PID patients and Number of PID patients per year estimated in the world, Africa and Europe. * Calculated on the basis of a prevalence of 3.72 per 100 000 (B. Gathmann, 2009). ** Calculated on the basis of an incidence of 10.3 per 100,000 persons per year (AY JOSHI, 2009). [1,2] If we do a projection based on the high prevalence in France (Gathmann B, 2009), we can expect to treat 37568 PIDs in Africa (see Table 1). If the incidence is 10.3 per 100,000 per year as reported by Joshi AY, 2009, we expect to treat 104019 PIDs per year. The overall number of PIDs reported at Casablanca in 2008 was 1049 (see Table 2). Regarding the number and distribution of PIDs in the 8 groups of the IUIS PID expert committee classification, we note that some diseases have specific distribution in Africa. For example, MHC class II deficiency and CGD are relatively common and predominant antibody deficiencies are less common. Several diseases cannot be diagnosed, in the absence of immunological and genetic investigations. Immunoglobulin substitution therapy is available in several countries, but only some countries have access to bone marrow transplantation. DIP MAROCTUNISIEALGERIEEGYPTE AF. DU SUD ASIDESID N=236N=397N=61N=204N=195N=1093N=7099 Incidence* A. Combined T and B cell immunodeficiency (CID) 21,6%23,4%18%25,4%13,84%21.4%9.2% SCID49%31.2% 61.5% 36.7%35.2% OMMEN SD2%8.6% 9.6% 6% MHC CLASS II deficiency27.5%36.6% 7.7% 22.2%8.5% B. Predominant antibody deficiencies 25,8%24,7%55,7%24,1%49,73%31%56.8% X-Linked Agamma (Bruton)19.7%21.4% 9.7% 10.8% CVID18%30.6% 28.6% 16.2%38.2% Hyper IgM14.8%22.4% 4.1% 9.7% 2.3% SUBCLASS IgG DEFICIENCY9.8%1% 4.1% 2,7% 12.6% SELECTIVE IgA DEFICIENCY29.5%16.3% 30.6% 14.5%13.5% C. Other well-defined immunodeficiency syndromes 32,2%23,2%9,8%16,66% 19%17.1% WAS 2.6%8.7% 14.7% 7.2% 23.8% ATAXIA TELANGIECTASIA35.5%70.7%100%41.1% 53.8%33.3% DIGEORGE ANOMALY13.2%4.3% 8.8% 8.2%20.3% HYPER IgE SD39.5%15.2% 26.4% 25.5% 9.9% D. Diseases of immune dysregulation 2,1%2% 1,6% 3.6% CHEDIACK HIGASHI SD 40%62.5% 41.1%5.8% GRISCELLI SD60% 50% 29.4%6.2% FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SD 37.5% 17.6% 34.4% E. Congenital defects of phagocytes 8,9%23,2%16,4%9,3%7,69%14.4% 9.8% SEVERE CONGENITAL NEUTROPENIA 19% 30% 4.5% KOSTMANN DISEASE9.5% 1.3%11.2% LAD4.8%18.5% 15.8% 13.4% 4.7% CGD28.6%44.6%70%68.4% 42.7% 29.6% MSMD14.3%21.7% 14.6%4.1% F. Defects in innate immunity0.4% 0,1%0.2% G. Autoinflammatory disorders4.2% 0,9% 1.1% H.Complement deficiencies4.7%1% 20%4,9%2.1% Table 2- PID distribution in several African countries and in Europe [3] National efforts and African cooperation are ongoing, supported by ESID but also national associations, the Jeffrey Modell Foundation and IPOPI. In this framework, an African registry project is in progress with the support of the ESID. References: 1.Gathmann B, France, 2009. 2.Joshi A.Y, USA, 2009. 3.Bousfiha AA et al, Epidémiologie et classification des déficits immunitaires primitifs, Rev Mar Mal Enf 2008, vol 18


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