Algerian Registry for Inborn Errors of Immunity in Children: Report of 887 Children (1985-2021)

Abdelghani Yagoubi; Azzeddine Tahiat; Nabila Souad Touri; Mohamed Samir Ladj; Ouardia Drali; Brahim Belaid; Ayda Mohand-Oussaid; Abdelhak Dehimi; Reda Belbouab; Yacine Ferhani; Souhila Melzi; Assia Guedouar; Saliha Hakem; Ouardia Khemici; Yacine Inouri; Yanis Meddour; Saadeddine Dib; Zohra Mansouri; Samir Iddir; Abderrahmane Boufersaoui; Houda Boudiaf; Abderrachid Bouhdjila; Ouardia Ibsaine; Hachemi Maouche; Djazia Dahlouk; Azzedine Mekki; Belkacem Bioud; Zair Bouzerar; Zoulikha Zeroual; Fadila Benhassine; Dahila Bekkat-Berkani; Soumeya Naamoune; Samir Sofiane Salah; Samia Chaib; Nabila Attal; Nadia Bensaadi; Nadira Bouchair; Nacira Cherif; Leila Kedji; Salih Bendeddouche; Mohamed Lamine Atif; Kamel Djenouhat; Nadia Kechout; Reda Djidjik; Keltoum Nafissa Benhalla; Leila Smati; Rachida Boukari

doi:10.1007/s10875-022-01330-5

Algerian Registry for Inborn Errors of Immunity in Children: Report of 887 Children (1985-2021)

J Clin Immunol. 2022 Nov;42(8):1660-1671. doi: 10.1007/s10875-022-01330-5. Epub 2022 Jul 15.

Authors

Abdelghani Yagoubi¹, Azzeddine Tahiat², Nabila Souad Touri³, Mohamed Samir Ladj⁴, Ouardia Drali⁵, Brahim Belaid⁶, Ayda Mohand-Oussaid⁷, Abdelhak Dehimi⁸, Reda Belbouab⁹, Yacine Ferhani⁹, Souhila Melzi¹⁰, Assia Guedouar¹¹, Saliha Hakem⁹, Ouardia Khemici¹², Yacine Inouri¹³, Yanis Meddour¹⁴, Saadeddine Dib¹⁵, Zohra Mansouri¹⁶, Samir Iddir¹⁷, Abderrahmane Boufersaoui¹⁸, Houda Boudiaf¹⁹, Abderrachid Bouhdjila²⁰, Ouardia Ibsaine¹⁶, Hachemi Maouche²¹, Djazia Dahlouk¹³, Azzedine Mekki⁵, Belkacem Bioud⁸, Zair Bouzerar¹⁰, Zoulikha Zeroual¹¹, Fadila Benhassine¹⁸, Dahila Bekkat-Berkani¹⁸, Soumeya Naamoune²², Samir Sofiane Salah²², Samia Chaib¹⁴, Nabila Attal²³, Nadia Bensaadi¹⁷, Nadira Bouchair²⁴, Nacira Cherif¹², Leila Kedji³, Salih Bendeddouche²⁵, Mohamed Lamine Atif²⁶, Kamel Djenouhat², Nadia Kechout²³, Reda Djidjik⁶, Keltoum Nafissa Benhalla⁷, Leila Smati¹⁸, Rachida Boukari²⁷

Affiliations

¹ Pediatric Gastroenterology and Digestive Investigations, Centre Algérois de Pédiatrie, 57 Lotissement F, Draria 16050, Algiers, Algeria.
² Department of Medical Biology, Rouiba Public Hospital, Rue Larbi Abdesalam 16017, Algiers, Algeria.
³ Department of Pediatrics, University Hospital Hassiba Ben Bouali, Rue Mohamed Boudiaf 09000, Blida, Algeria.
⁴ Department of Pediatrics, Djilali Belkhenchir Public Hospital, El-Biar, Algiers, Algeria.
⁵ Department of Pediatrics "B", University Hospital Nafissa Hamoud, Rue Boudjemaa Moghni, Hussein Dey 16040, Algiers, Algeria.
⁶ Department of Immunology, University Hospital Issaad Hassani, Beni Messous, 16206, Algiers, Algeria.
⁷ Department of Pediatrics "A", University Hospital Issaad Hassani, Beni Messous, 16206, Algiers, Algeria.
⁸ Pediatric Division, University Hospital Abdenour Saadna, 19000, Setif, Algeria.
⁹ Department of Pediatrics, University Hospital Mustapha Bacha, Place 1er Mai 1945, Sidi Mhamed, 16024, Algiers, Algeria.
¹⁰ Department of Pediatrics, University Hospital Lamine Debaghine, Said Touati, Bab el Oued, 16009, BdAlgiers, Algeria.
¹¹ Department of Pediatrics "A", University Hospital Nafissa Hamoud, Rue Boudjemaa Moghni, Hussein Dey 16040, Algiers, Algeria.
¹² Department of Pediatrics "B", University Hospital Issaad Hassani, Beni Messous, 16206, Algiers, Algeria.
¹³ Department of Pediatrics, Central Army Hospital, Ain Naadja 16205, Algiers, Algeria.
¹⁴ Department of Immunology, Central Army Hospital, Ain Naadja 16205, Algiers, Algeria.
¹⁵ Department of Pediatrics, University Hospital Dr Tidjani Damerdji, 05, Bd Mohammed V, Tlemcen, Algeria.
¹⁶ Department of Pediatrics, Ain Taya Public Hospital, Ain Taya, Algiers, Algeria.
¹⁷ Department of Pediatrics, University Hospital Nedir Mohamed, Tizi Ouzou, Algeria.
¹⁸ Department of Pediatrics, Bologhine Ibn Ziri Public Hospital, Rue Aboumoussa El Achaari Hammamet 16060, Algiers, Algeria.
¹⁹ Department of Pediatric Oncology, University Hospital Mustapha Bacha, Place 1er Mai 1945, Sidi Mhamed, 16024, Algiers, Algeria.
²⁰ Department of Pediatrics "B", University Hospital Ben Badis, Constantine, Algeria.
²¹ Department of Pediatrics, Hassen Badi Public Hospital, El-Harrach, Algiers, Algeria.
²² Department of Immunology, University Hospital Mustapha Bacha, Place 1er Mai 1945, Sidi Mhamed, 16024, Algiers, Algeria.
²³ Department of Immunology, DelyBrahim, Institut Pasteur d'Alger, Algiers, Algeria.
²⁴ Department of Pediatrics, Clinique Sainte Thérèse, Rue Calama, Annaba, Algeria.
²⁵ Department of Pediatrics, Mother and Child Specialized Hospital, Tlemcen, Algeria.
²⁶ Department of Epidemiology and Preventive Medicine, University Hospital Douera, Douera, Algiers, Algeria.
²⁷ Department of Pediatrics, University Hospital Mustapha Bacha, Place 1er Mai 1945, Sidi Mhamed, 16024, Algiers, Algeria. rachida_boukari@yahoo.fr.

PMID: 35838820
DOI: 10.1007/s10875-022-01330-5

Abstract

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.

Keywords: Algeria; Children; Inborn errors of immunity; Registry.

MeSH terms

Agammaglobulinemia* / epidemiology
Algeria / epidemiology
Child
Humans
Immunologic Deficiency Syndromes* / diagnosis
Immunologic Deficiency Syndromes* / epidemiology
Immunologic Deficiency Syndromes* / genetics
Male
Primary Immunodeficiency Diseases*
Registries