Inborn Errors of Immunity in Algerian Children and Adults: A Single-Center Experience Over a Period of 13 Years (2008–2021)
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 , Casanova Jean Laurent , Puel Anne , Lau Yu Lung , Cherif Nacira , Ladj Samir , Smati Leila , Boukari Rachida , Benhalla Nafissa , Djidjik Reda 
Published: April 2022

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.