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Introduction: Inborn errors of immunity (IEI) represent a heterogeneous large group of genetic disorders characterized by susceptibility of affected individuals to recurrent infections, autoimmune/inflammatory diseases, allergy, and malignancy. We aimed to report for the first time the Algerian registry for IEI in children., Methods: We described the characteristics of IEI in Algerian children from the data collected in the Algerian registry for IEI between 1985 and 2021., Results: Over a period of 37 years, we included 887 children (530 male, 59.6%) with a mean age at diagnosis of 3.23 years and a mean diagnosis delay of 2 years. 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 immunodeficiencies (CID) (35.5%), followed by predominantly antibody deficiencies (24.5%) and CID with syndromic features (18.3%). The most predominant diseases were severe CID (134 cases), MHC II deficiency (99 cases), agammaglobulinemia (82 cases), common variable immunodeficiency (78 cases), hyper IgE syndromes (61 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 first report of the Algerian registry for IEI in children. Data is globally similar to that of the Middle East and North African (MENA) registries with high consanguinity, predominance of CID, and significant mortality. This registry highlights the weak points that should be improved in order to provide better patient care., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Inborn errors of immunity (IEI) predispose patients to various infectious and non-infectious complications. Thanks to the development and expanding use of flow cytometry and increased awareness, the diagnostic rate of IEI has markedly increased in Algeria the last decade., Aim: This study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes., Methods: We collected and analyzed retrospectively the demographic data, clinical manifestations, immunologic, genetic data, and outcome of Algerian IEI patients - diagnosed in the department of medical immunology of Beni Messous university hospital center, Algiers, from 2008 to 2021., Results: Eight hundred and seven patients with IEI (482 males and 325 females) were enrolled, 9.7% of whom were adults. Consanguinity was reported in 50.3% of the cases and a positive family history in 32.34%. The medium age at disease onset was 8 months and at diagnosis was 36 months. The median delay in diagnosis was 16 months. Combined immunodeficiencies were the most frequent (33.8%), followed by antibody deficiencies (24.5%) and well-defined syndromes with immunodeficiency (24%). Among 287 patients tested for genetic disorders, 129 patients carried pathogenic mutations; 102 having biallelic variants mostly in a homozygous state (autosomal recessive disorders). The highest mortality rate was observed in patients with combined immunodeficiency (70.1%), especially in patients with severe combined immunodeficiency (SCID), Omenn syndrome, or Major Histocompatibility Complex (MHC) class II deficiency., Conclusion: The spectrum of IEI in Algeria is similar to that seen in most countries of the Middle East and North Africa (MENA) region, notably regarding the frequency of autosomal recessive and/or combined immunodeficiencies., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Belaid, Lamara Mahammed, Drali, Oussaid, Touri, Melzi, Dehimi, Berkani, Merah, Larab, Allam, Khemici, Kirane, Boutaba, Belbouab, Bekkakcha, Guedouar, Chelali, Baamara, Noui, Baaziz, Rezak, Azzouz, Aichaoui, Moktefi, Benhatchi, Oussalah, Benaissa, Laredj, Bouchetara, Adria, Habireche, Tounsi, Dahmoun, Touati, Boucenna, Bouferoua, Sekfali, Bouhafs, Aboura, Kherra, Inouri, Dib, Medouri, Khelfaoui, Redjedal, Zelaci, Yahiaoui, Medjadj, Touhami, Kadi, Amireche, Frada, Houasnia, Benarab, Boubidi, Ferhani, Benalioua, Sokhal, Benamar, Aggoune, Hadji, Bellouti, Rahmoune, Boutrid, Okka, Ammour, Saadoune, Amroun, Belhadj, Ghanem, Abbaz, Boudrioua, Zebiche, Ayad, Hamadache, Ouaras, Achour, Bouchair, Boudiaf, Bekkat-Berkani, Maouche, Bouzrar, Aissat, Ibsaine, Bioud, Kedji, Dahlouk, Bensmina, Radoui, Bessahraoui, Bensaadi, Mekki, Zeroual, Chan, Leung, Tebaibia, Ayoub, Mekideche, Gharnaout, Casanova, Puel, Lau, Cherif, Ladj, Smati, Boukari, Benhalla and Djidjik.)