[Primary immunodeficiency in children in Casablanca: a 73 cases report]

Primary immunodeficiencies [PID] seem to have two particularities in the Maghreb countries: a higher frequency than in the occidental countries and a higher proportion of deficiency of HLA class II antigens. Patients and method: a four-year study reports 73 PID cases between 1997 March and 2001 February in the Clinical Immunology Unit of the Casablanca Children Hospital. These 73 cases were diagnosed, according to the WHO criteria, among 358 children with recurrent and/or severe infections. They had an immunological assessment: tuberculin IDR, blood cells, serum immunoglobulin A G and M, lymphocytes classes. we found 18 combined immunodeficiency including 5 deficiencies of HLA class II antigens, 18 antibody defects, 17 complex immunodeficiencies including 11 ataxia-telangiectasia syndromes, 13 PID associated to an other disorder including 4 hyper IgM syndrome, 6 phagocyte disorders and one complement defect. Because of their high cost, the intravenous immunoglobulins were used in only 29 children and the bone marrow transplantation is not available in our country. Twenty six children died in this study. Comments: as in Tunisia, our study confirms the high frequency of combined and complex PID in comparison to the occidental studies, and we have to develop the PID diagnostic and treatment resources in our country

[Specific immunity hereditary deficits]

Hereditary anomalies of specific immunity include a wide range of different deficits. Some anomalies consist of a predominant deficiency of antibody production such as sex-linked hypogammaglobulinaemia. Other anomalies consist of dissociated deficits of immunity: IgA and IgG deficits with raised IgM, isolated serum IgM deficit; IgA deficit, IgG[2] and IgG[4] isotype; deficit or hypogammaglobulinaemia of infancy. Certain deficiencies predominantly affect cell-mediated immunity, such as Di George's syndrome [hypoplastic thymus] or deficit purine nucleoside phosphorylase. Other deficits simultaneously affect humoral immunity and cell-mediated immunity. These are severe immune deficiencies [SID]: alymphocytosis with agammaglobulinaemia and B cells present or adenosine desaminase deficiency [A.D.A]. The diagnosis of SID is based on a high susceptibility to infections or graft versus host [GvH] reactions. Treatment consists of bone marrow graft with an HLA identical or semi-identical marrow. Lastly, certain immune deficiencies are associated with other complex anomalies: ataxia telanglectasies, Wiskott-Aldrich syndrome, achondroplastic dwarfism, hypopigmentation, chronic mucocutaneous candidiasis, transcobalamine II deficiency. The antenatal diagnosis of immune deficiencies is currently under investigation