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

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.

Prevalence rate of exercise-induced bronchoconstriction in Annaba (Algeria) schoolchildren.

BACKGROUND: We studied the prevalence rate of exercise-induced bronchoconstriction (EIB) in Annaba schoolchildren. No previous assessment of this syndrome had ever been done in Algeria. METHODS: EIB was evaluated using the 6 min free running test (6MFRT) on 286 children, 87.4% of whom were 10-12 years old. They performed the test in the morning, during the autumn-winter season. Peak expiratory flow rate (PEFR) was measured before, and then 5 and 10 min after the 6MFRT, a 15% or more decrease in post-exercise PEFR being defined as significant. RESULTS: EIB was much more frequent in asthmatic than in nonasthmatic children (47.0% vs. 13.9%, p < .001) and the drop in PEFR was more marked among the former. EIB was observed more often in a cool temperature (<8°C). There were relatively more children who were either eutrophic or overweight among those presenting an EIB. Past histories of nocturnal wheezing and rhinoconjunctivitis presented respectively the best specificity (96.7%) and sensitivity (84.8%). Children of unemployed workers presented the highest occurrence of EIB. Passive smoking appeared as the only factor being related to EIB among the indoor pollutants. The prevalence rate of asthma (6.7%) was higher than in a previous cross-sectional epidemiological survey study performed in the Maghreb. The prevalence rate of EIB (13.9%) was situated in the upper range of the results given worldwide. CONCLUSIONS: These features of a fairly high bronchial hyperresponsiveness could be facilitated by the polluted environment of the city.