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

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

Algeria Children Inborn errors of immunity Registry

Journal

Journal of clinical immunology

ISSN: 1573-2592

Titre abrégé: J Clin Immunol

Pays: Netherlands

ID NLM: 8102137

Informations de publication

Date de publication:
11 2022

Historique:

received: 09 02 2022

accepted: 03 07 2022

pubmed: 16 7 2022

medline: 30 11 2022

entrez: 15 7 2022

Statut: ppublish

Résumé

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. We described the characteristics of IEI in Algerian children from the data collected in the Algerian registry for IEI between 1985 and 2021. 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. 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.

Identifiants

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

pubmed: 35838820

doi: 10.1007/s10875-022-01330-5

pii: 10.1007/s10875-022-01330-5

doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Pagination

1660-1671

Informations de copyright

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