Introduction: Inborn errors of immunity (IEI) represent a heterogeneous large group of genetic disorders characterized by susceptibility of affected individuals to recurrent infections, autoimmune/in ammatory diseases, allergies and malignancy. We aim to report for the rst time the Algerian registry for IEI in children.Methods: We describe the characteristics of IEI in Algerian children from the data collected in the Algerian registry for IEI over a long period of 37 years.Results: Between 1985 and 2021, we included 887 children (530 male, 59.6%) with a mean age at diagnosis of 3.23y and a mean diagnosis delay of 2y. The prevalence rate was estimated at 1.97/100,000 inhabitants or 5.91/100,000 children. The parental consanguinity was found in 52.6%. The most prevalent category was combined immunode ciencies (CID) (35.5%) followed by predominantly antibody de ciencies (24.4%) and CID with syndromic features (17.9%). The most predominant diseases were severe CID (120 cases), MHC II de ciency (99 cases), agammaglobulinemia (81 cases), common variable immunode ciency (74 cases), hyper IgE syndromes (60 patients), ataxia telangiectasia (46 patients), Wiskott Aldrich syndrome (40 patients) and chronic granulomatous disease (39 cases). The clinical presentation was dominated by lower respiratory tract infections (69%), failure to thrive (38.3%) and chronic diarrhea (35.2%). Genetic analysis was performed in 156 patients (17.6%). The global mortality rate was 28.4% mainly caused by CID.Conclusion: This is the rst report of the Algerian registry for IEI in children. Data is globally similar to that of Middle East and North African (MENA) registries with high consanguinity, predominance of CID, and signi cant mortality. This registry highlights the weak points that should be improved in order to provide better patient care.
