Inborn Errors of Immunity in Algerian Children and Adults: A Single-Center Experience Over a Period of 13 Years (2008-2021)

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. This study aimed to describ...

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Published in:	Frontiers in immunology Vol. 13; p. 900091
Main Authors:	Belaid, Brahim, Lamara Mahammed, Lydia, Drali, Ouardia, Oussaid, Aida Mohand, Touri, Nabila Souad, Melzi, Souhila, Dehimi, Abdelhak, Berkani, Lylia Meriem, Merah, Fatma, Larab, Zineb, Allam, Ines, Khemici, Ouarda, Kirane, Sonya Yasmine, Boutaba, Mounia, Belbouab, Reda, Bekkakcha, Hadjira, Guedouar, Assia, Chelali, Abdelhakim, Baamara, Brahim, Noui, Djamila, Baaziz, Hadda, Rezak, Radia, Azzouz, Sidi Mohamed, Aichaoui, Malika, Moktefi, Assia, Benhatchi, Redha Mohamed, Oussalah, Meriem, Benaissa, Naila, Laredj, Amel, Bouchetara, Assia, Adria, Abdelkader, Habireche, Brahim, Tounsi, Noureddine, Dahmoun, Fella, Touati, Rabah, Boucenna, Hamza, Bouferoua, Fadila, Sekfali, Lynda, Bouhafs, Nadjet, Aboura, Rawda, Kherra, Sakina, Inouri, Yacine, Dib, Saadeddine, Medouri, Nawel, Khelfaoui, Noureddine, Redjedal, Aicha, Zelaci, Amara, Yahiaoui, Samah, Medjadj, Sihem, Touhami, Tahar Khelifi, Kadi, Ahmed, Amireche, Fouzia, Frada, Imane, Houasnia, Shahrazed, Benarab, Karima, Boubidi, Chahynez, Ferhani, Yacine, Benalioua, Hayet, Sokhal, Samia, Benamar, Nadia, Aggoune, Samira, Hadji, Karima, Bellouti, Asma, Rahmoune, Hakim, Boutrid, Nada, Okka, Kamelia, Ammour, Assia, Saadoune, Houssem, Amroun, Malika, Belhadj, Hayet, Ghanem, Amina, Abbaz, Hanane, Boudrioua, Sana, Zebiche, Besma, Ayad, Assia, Hamadache, Zahra, Ouaras, Nassima, Achour, Nassima, Bouchair, Nadira, Boudiaf, Houda, Bekkat-Berkani, Dahila, Maouche, Hachemi, Bouzrar, Zahir, Aissat, Lynda, Ibsaine, Ouardia, Bioud, Belkacem, Kedji, Leila, Dahlouk, Djazia, Bensmina, Manoubia, Radoui, Abdelkarim, Bessahraoui, Mimouna, Bensaadi, Nadia, Mekki, Azzeddine, Zeroual, Zoulikha, Chan, Koon-Wing, Leung, Daniel, Tebaibia, Amar, Ayoub, Soraya, Mekideche, Dalila, Gharnaout, Merzak
Format:	Journal Article
Language:	English
Published:	Switzerland Frontiers Media S.A 21-04-2022
Subjects:	Algeria clinical features epidemiology Immunology inborn errors of immunity molecular diagnosis primary immunodeficiency epidemiology primary immunodeficiency diagnosis Algeria inborn errors of immunity clinical features molecular diagnosis
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Summary:	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. This study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes. 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. 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. 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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 Edited by: Waleed Al-Herz, Kuwait University, Kuwait Reviewed by: Kathleen Sullivan, Children’s Hospital of Philadelphia, United States; Javier Chinen, Baylor College of Medicine, United States This article was submitted to Primary Immunodeficiencies, a section of the journal Frontiers in Immunology
ISSN:	1664-3224 1664-3224
DOI:	10.3389/fimmu.2022.900091