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

Adult Algeria / epidemiology Child Female Humans Immunologic Deficiency Syndromes / genetics Male Primary Immunodeficiency Diseases / genetics Retrospective Studies Severe Combined Immunodeficiency
Algeria clinical features diagnosis epidemiology inborn errors of immunity molecular diagnosis primary immunodeficiency

Journal

Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960

Informations de publication

Date de publication:
2022
Historique:
received: 19 03 2022
accepted: 25 03 2022
entrez: 9 5 2022
pubmed: 10 5 2022
medline: 11 5 2022
Statut: epublish

Résumé

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.

Sections du résumé

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.

Identifiants

DOI: 10.3389/fimmu.2022.900091 PMID: 35529857 PMC: PMC9069527
pubmed: 35529857
doi: 10.3389/fimmu.2022.900091
pmc: PMC9069527
doi:

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

900091

Subventions

Organisme : NIAID NIH HHS
ID : R01 AI127564
Pays : United States
Organisme : NIAID NIH HHS
ID : R01 AI095983
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001866
Pays : United States
Organisme : Howard Hughes Medical Institute
Pays : United States

Informations de copyright

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.

Déclaration de conflit d'intérêts

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.

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Auteurs

Brahim Belaid (B)

Department of Medical Immunology, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Lydia Lamara Mahammed (L)

Department of Medical Immunology, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Ouardia Drali (O)

Department of Pediatrics B, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Aida Mohand Oussaid (AM)

Department of Pediatrics A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Nabila Souad Touri (NS)

Department of Pediatrics, Blida University Hospital Center, University of Blida, Blida, Algeria.

Souhila Melzi (S)

Department of Pediatrics, Bab El Oued University Hospital Center, University of Algiers 1, Algiers, Algeria.

Abdelhak Dehimi (A)

Department of Pediatrics, Setif University Hospital Center, University of Setif 1, Setif, Algeria.

Lylia Meriem Berkani (LM)

Department of Medical Immunology, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Fatma Merah (F)

Department of Medical Immunology, Beni Messous University Hospital Center, Algiers, Algeria.

Zineb Larab (Z)

Department of Medical Immunology, Beni Messous University Hospital Center, Algiers, Algeria.

Ines Allam (I)

Department of Medical Immunology, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Ouarda Khemici (O)

Department of Pediatrics B, Beni Messous University Hospital Center, Algiers, Algeria.

Sonya Yasmine Kirane (SY)

Department of Pediatrics B, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Mounia Boutaba (M)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Reda Belbouab (R)

Department of Pediatrics, Mustapha Pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Hadjira Bekkakcha (H)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Assia Guedouar (A)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Abdelhakim Chelali (A)

Department of Pediatrics, Djelfa Public Hospital Institution, Djelfa, Algeria.

Brahim Baamara (B)

Department of Pediatrics, Djelfa Public Hospital Institution, Djelfa, Algeria.

Djamila Noui (D)

Department of Pediatrics, Batna University Hospital center, University of Batna, Batna, Algeria.

Hadda Baaziz (H)

Department of Pediatrics, Batna University Hospital center, University of Batna, Batna, Algeria.

Radia Rezak (R)

Department of Pediatric Gastroenterology and Nutrition, Canastel Children's Hospital, Oran, Algeria.

Sidi Mohamed Azzouz (SM)

Department of Pediatric Gastroenterology and Nutrition, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Malika Aichaoui (M)

Department of Pediatric Pneumo-Allergology, Canastel Children's Hospital, Oran, Algeria.

Assia Moktefi (A)

Department of Pediatric Pneumo-Allergology, Canastel Children's Hospital, Oran, Algeria.

Redha Mohamed Benhatchi (RM)

Department of Pediatric Pneumo-Allergology, Canastel Children's Hospital, Oran, Algeria.

Meriem Oussalah (M)

Department of Pediatric Pneumo-Allergology, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Naila Benaissa (N)

Department of Children's Infectious Diseases, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Amel Laredj (A)

Department of Children's Infectious Diseases, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Assia Bouchetara (A)

Department of Children's Infectious Diseases, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Abdelkader Adria (A)

Department of Pediatric Hematology, Canastel Children's Hospital, Oran, Algeria.

Brahim Habireche (B)

Department of Pediatrics, El Bayadh Public Hospital Institution, EL Bayadh, Algeria.

Noureddine Tounsi (N)

Department of Pediatrics, El Bayadh Public Hospital Institution, EL Bayadh, Algeria.

Fella Dahmoun (F)

Department of Pediatrics, Bejaia University Hospital Center, University of Bejaia, Bejaia, Algeria.

Rabah Touati (R)

Department of Pediatrics, Bejaia University Hospital Center, University of Bejaia, Bejaia, Algeria.

Hamza Boucenna (H)

Department of Pediatrics A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Fadila Bouferoua (F)

Department of Pediatrics A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Lynda Sekfali (L)

Department of Pediatrics A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Nadjet Bouhafs (N)

Department of Pediatrics, Bab El Oued University Hospital Center, University of Algiers 1, Algiers, Algeria.

Rawda Aboura (R)

Department of Pediatrics, Bab El Oued University Hospital Center, University of Algiers 1, Algiers, Algeria.

Sakina Kherra (S)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Yacine Inouri (Y)

Department of Pediatrics, Central Hospital of the Army, University of Algiers 1, Algiers, Algeria.

Saadeddine Dib (S)

Department of Pediatrics, Mother & Child Hospital of Tlemcen, University of Tlemcen, Tlemcen, Algeria.

Nawel Medouri (N)

Department of Pediatrics, Saida Public Hospital Institution, Saida, Algeria.

Noureddine Khelfaoui (N)

Department of Pediatrics, Saida Public Hospital Institution, Saida, Algeria.

Aicha Redjedal (A)

Department of Pediatrics, Saida Public Hospital Institution, Saida, Algeria.

Amara Zelaci (A)

Department of Pediatrics, El Oued Public Hospital Institution, El Oued, Algeria.

Samah Yahiaoui (S)

Department of Pediatrics, Barika Public Hospital Institution, Batna, Algeria.

Sihem Medjadj (S)

Department of Pediatrics, Ghardaia Public Hospital Institution, Ghardaia, Algeria.

Tahar Khelifi Touhami (TK)

Private Practitioner, Constantine, Algeria.

Ahmed Kadi (A)

Department of Pneumology A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Fouzia Amireche (F)

Department of Pediatrics, Mother & Child Hospital of EL Mansourah, University of Constantine 3, Constantine, Algeria.

Imane Frada (I)

Department of Pediatrics, Biskra Public Hospital Institution, Biskra, Algeria.

Shahrazed Houasnia (S)

Department of Pediatrics, El Harrouche Public Hospital Institution, Skikda, Algeria.

Karima Benarab (K)

Department of Pediatrics, Tizi Ouzou University Hospital Center, University of Tizi Ouzou, Tizi Ouzou, Algeria.

Chahynez Boubidi (C)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Yacine Ferhani (Y)

Department of Pediatrics, Mustapha Pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Hayet Benalioua (H)

Department of Pediatrics, Mustapha Pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Samia Sokhal (S)

Department of Pediatrics, Mustapha Pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Nadia Benamar (N)

Department of Pediatrics, Tighennif Public Hospital Institution, Mascara, Algeria.

Samira Aggoune (S)

Department of Pediatrics, El-Harrach Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Karima Hadji (K)

Department of Pediatrics, Ain Oulmene Public Hospital Institution, Setif, Algeria.

Asma Bellouti (A)

Department of Pediatrics, Ain Azel Public Hospital Institution, Setif, Algeria.

Hakim Rahmoune (H)

Department of Pediatrics, Setif University Hospital Center, University of Setif 1, Setif, Algeria.

Nada Boutrid (N)

Department of Pediatrics, Setif University Hospital Center, University of Setif 1, Setif, Algeria.

Kamelia Okka (K)

Department of Pediatrics, Setif University Hospital Center, University of Setif 1, Setif, Algeria.

Assia Ammour (A)

Department of Pediatrics, Mother & Child Hospital of Touggourt, Touggourt, Algeria.

Houssem Saadoune (H)

Department of Pneumology, Mila Public Hospital Institution, Mila, Algeria.

Malika Amroun (M)

Department of Pediatrics, Central Hospital of the Army, University of Algiers 1, Algiers, Algeria.

Hayet Belhadj (H)

Department of Pediatrics, Central Hospital of the Army, University of Algiers 1, Algiers, Algeria.

Amina Ghanem (A)

Department of Pediatrics, Khenchela Public Hospital Institution, Khenchela, Algeria.

Hanane Abbaz (H)

Department of Pediatrics, Khenchela Public Hospital Institution, Khenchela, Algeria.

Sana Boudrioua (S)

Department of Pediatrics, El Khroub Public Hospital Institution, Constantine, Algeria.

Besma Zebiche (B)

Department of Pediatrics, Kolea Public Hospital Institution, Tipaza, Algeria.

Assia Ayad (A)

Department of Pediatrics, Kolea Public Hospital Institution, Tipaza, Algeria.

Zahra Hamadache (Z)

Department of Pediatrics, Kolea Public Hospital Institution, Tipaza, Algeria.

Nassima Ouaras (N)

Department of Infectious Diseases, EL Kettar Specialized Hospital, University of Algiers 1, Algiers, Algeria.

Nassima Achour (N)

Department of Infectious Diseases, EL Kettar Specialized Hospital, University of Algiers 1, Algiers, Algeria.

Nadira Bouchair (N)

Department of Pediatrics, Annaba University Hospital Center, University of Annaba, Annaba, Algeria.

Houda Boudiaf (H)

Department of Pediatric Oncology, Mustapha pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Dahila Bekkat-Berkani (D)

Department of Pediatrics, Bologhine Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Hachemi Maouche (H)

Department of Pediatrics, El-Harrach Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Zahir Bouzrar (Z)

Department of Pediatrics, Bab El Oued University Hospital Center, University of Algiers 1, Algiers, Algeria.

Lynda Aissat (L)

Department of Pediatrics, Mother & Child Hospital of Tipaza, University of Blida, Algiers, Algeria.

Ouardia Ibsaine (O)

Department of Pediatrics, Ain Taya Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Belkacem Bioud (B)

Department of Pediatrics, Setif University Hospital Center, University of Setif 1, Setif, Algeria.

Leila Kedji (L)

Department of Pediatrics, Blida University Hospital Center, University of Blida, Blida, Algeria.

Djazia Dahlouk (D)

Department of Pediatrics, Central Hospital of the Army, University of Algiers 1, Algiers, Algeria.

Manoubia Bensmina (M)

Department of Pediatrics B, Douera University Hospital Center, University of Blida, Algiers, Algeria.

Abdelkarim Radoui (A)

Department of Pediatric Pneumo-Allergology, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Mimouna Bessahraoui (M)

Department of Pediatric Gastroenterology and Nutrition, Canastel Children's Hospital, University of Oran, Oran, Algeria.

Nadia Bensaadi (N)

Department of Pediatrics, Tizi Ouzou University Hospital Center, University of Tizi Ouzou, Tizi Ouzou, Algeria.

Azzeddine Mekki (A)

Department of Pediatrics B, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Zoulikha Zeroual (Z)

Department of Pediatrics A, Hussein Dey University Hospital Center, University of Algiers 1, Algiers, Algeria.

Koon-Wing Chan (KW)

Department of Pediatrics and Adolescent Medicine, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong SAR, China.

Daniel Leung (D)

Department of Pediatrics and Adolescent Medicine, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong SAR, China.

Amar Tebaibia (A)

Department of Internal Medicine, El Biar Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Soraya Ayoub (S)

Department of Internal Medicine, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Dalila Mekideche (D)

Department of Pneumology B, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Merzak Gharnaout (M)

Department of Pneumology A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Jean Laurent Casanova (JL)

Laboratory of Human Genetics of Infectious Diseases, Necker Hospital for Sick Children, INSERM UMR 1163, Paris, France.
Imagine Institute, University of Paris, Paris, France.
St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller University, New York, NY, United States.
Howard Hughes Medical Institute, New York, NY, United States.

Anne Puel (A)

Laboratory of Human Genetics of Infectious Diseases, Necker Hospital for Sick Children, INSERM UMR 1163, Paris, France.
Imagine Institute, University of Paris, Paris, France.
St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller University, New York, NY, United States.

Yu Lung Lau (YL)

Department of Pediatrics and Adolescent Medicine, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong SAR, China.

Nacira Cherif (N)

Department of Pediatrics B, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Samir Ladj (S)

Department of Pediatrics, El Biar Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Leila Smati (L)

Department of Pediatrics, Bologhine Public Hospital Institution, University of Algiers 1, Algiers, Algeria.

Rachida Boukari (R)

Department of Pediatrics, Mustapha Pacha University Hospital Center, University of Algiers 1, Algiers, Algeria.

Nafissa Benhalla (N)

Department of Pediatrics A, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

Reda Djidjik (R)

Department of Medical Immunology, Beni Messous University Hospital Center, University of Algiers 1, Algiers, Algeria.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Régions géographiques › Afrique › Afrique du Nord › Algérie › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Maladies du système immunitaire › Déficits immunitaires › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Maladies du système immunitaire › Déficits immunitaires › Maladies d'immunodéficience primaire › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études rétrospectives › Inborn Errors of Immunity in Algerian Children...
questionsmedicales.fr › Maladies du système immunitaire › Déficits immunitaires › Maladies d'immunodéficience primaire › Immunodéficience combinée grave › Inborn Errors of Immunity in Algerian Children...