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. 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.
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.
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.
Authors: A A Bousfiha; L Jeddane; N El Hafidi; N Benajiba; N Rada; J El Bakkouri; A Kili; S Benmiloud; I Benhsaien; I Faiz; O Maataoui; Z Aadam; A Aglaguel; L Ait Baba; Z Jouhadi; R Abilkassem; M Bouskraoui; M Hida; J Najib; H Salih Alj; F Ailal Journal: J Clin Immunol Date: 2014-03-12 Impact factor: 8.317
Authors: Markus G Seidel; Gerhard Kindle; Benjamin Gathmann; Isabella Quinti; Matthew Buckland; Joris van Montfrans; Raphael Scheible; Stephan Rusch; Lukas M Gasteiger; Bodo Grimbacher; Nizar Mahlaoui; Stephan Ehl Journal: J Allergy Clin Immunol Pract Date: 2019-02-15