1. Inborn Errors of Immunity in Algerian Children and Adults: A Single-Center Experience Over a Period of 13 Years (2008–2021)
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Brahim Belaid, Lydia Lamara Mahammed, Ouardia Drali, Aida Mohand Oussaid, Nabila Souad Touri, Souhila Melzi, Abdelhak Dehimi, Lylia Meriem Berkani, Fatma Merah, Zineb Larab, Ines Allam, Ouarda Khemici, Sonya Yasmine Kirane, Mounia Boutaba, Reda Belbouab, Hadjira Bekkakcha, Assia Guedouar, Abdelhakim Chelali, Brahim Baamara, Djamila Noui, Hadda Baaziz, Radia Rezak, Sidi Mohamed Azzouz, Malika Aichaoui, Assia Moktefi, Redha Mohamed Benhatchi, Meriem Oussalah, Naila Benaissa, Amel Laredj, Assia Bouchetara, Abdelkader Adria, Brahim Habireche, Noureddine Tounsi, Fella Dahmoun, Rabah Touati, Hamza Boucenna, Fadila Bouferoua, Lynda Sekfali, Nadjet Bouhafs, Rawda Aboura, Sakina Kherra, Yacine Inouri, Saadeddine Dib, Nawel Medouri, Noureddine Khelfaoui, Aicha Redjedal, Amara Zelaci, Samah Yahiaoui, Sihem Medjadj, Tahar Khelifi Touhami, Ahmed Kadi, Fouzia Amireche, Imane Frada, Shahrazed Houasnia, Karima Benarab, Chahynez Boubidi, Yacine Ferhani, Hayet Benalioua, Samia Sokhal, Nadia Benamar, Samira Aggoune, Karima Hadji, Asma Bellouti, Hakim Rahmoune, Nada Boutrid, kamelia Okka, Assia Ammour, Houssem Saadoune, Malika Amroun, Hayet Belhadj, Amina Ghanem, Hanane Abbaz, Sana Boudrioua, Besma Zebiche, Assia Ayad, Zahra Hamadache, Nassima Ouaras, Nassima Achour, Nadira Bouchair, Houda Boudiaf, Dahila Bekkat-Berkani, Hachemi Maouche, Zahir Bouzrar, Lynda Aissat, Ouardia Ibsaine, Belkacem Bioud, Leila Kedji, Djazia Dahlouk, Manoubia Bensmina, Abdelkarim Radoui, Mimouna Bessahraoui, Nadia Bensaadi, Azzeddine Mekki, Zoulikha Zeroual, Koon-Wing Chan, Daniel Leung, Amar Tebaibia, Soraya Ayoub, Dalila Mekideche, Merzak Gharnaout, Jean Laurent Casanova, Anne Puel, Yu Lung Lau, Nacira Cherif, Samir Ladj, Leila Smati, Rachida Boukari, Nafissa Benhalla, and Reda Djidjik
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primary immunodeficiency ,inborn errors of immunity ,Algeria ,epidemiology ,molecular diagnosis ,clinical features ,Immunologic diseases. Allergy ,RC581-607 - Abstract
BackgroundInborn 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.AimThis study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes.MethodsWe 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.ResultsEight 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.ConclusionThe 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.
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- 2022
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