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.

Does omeprazole improve asthma-control in poorly-controlled asthmatic children with gastro-esophageal reflux.

OBJECTIVES: The role of gastro-esophageal reflux disease (GERD) in poorly controlled asthma is often mentioned, but published studies have presented discordant results. Our main objective was to assess the effectiveness of GERD treatment in controlling asthma in children. METHODS: We conducted a prospective study including poorly controlled asthmatic children aged 4 to 16 years. We checked the presence of acid reflux using pH monitoring. Patients with GERD were randomized into two groups; one received omeprazole for 6 months and the control group was not treated. The outcome was the score of the children asthma control test at the end of 6 months. The acid suppression was checked at the end of treatment with pH monitoring. After treatment, children with persistent acid reflux received high PPI doses and therefore were reevaluated 6 months later. RESULTS: We included 102 children with poorly controlled asthma among which 59 (57.8%) had acid reflux. Gastroesophageal reflux (GER) was significantly more common in boys (p = 0.04). Treatment with omeprazole in sufficient doses improved the control of asthma in 5 children out of 6 (84.8 vs 11.5; p<.0001). Three factors appeared to be statistically associated with asthma control improvement after PPI therapy: male sex (p=.04), normal birth weight (p=.05) and a positive Prick-test (p=.05). These factors were not confirmed or were not sufficiently precise in multivariate analysis. The likelihood of a causal relationship between acid reflux and asthma, difficult to highlight with pH monitoring, was poor. CONCLUSIONS: This study confirmed the high prevalence of GER in poorly controlled asthmatic children and showed the possible benefit of an efficient GER treatment in improving asthma control.

Children hypertension in Northern Africa.

OBJECTIVES: To assess factors associated with prehypertension and hypertension among children in North Africa. METHODS: An epidemiological observational, school- and college-based study among 3562 healthy children and adolescents to assess factors associated with blood pressure categories (normal, prehypertensive, hypertensive), including perinatal (gestational age, birth weight, breastfeeding) and current lifestyle characteristics (body mass index, time spent watching a screen and time spent exercising). RESULTS: Prevalence of hypertension increased with age from 8.7% between 6-10 years to 14.7% between 11-15 years, and 15.6% above 15 years. Prevalence of prehypertension and hypertension increased with body mass index from 9.9% and 11.5% among children not overweight to 15.6% (RR 1.58, 95% CI 1.24-2.02, P<0.001) and 17.2% (RR 1.50, 95% CI 1.22-1.85, P<0.001) among those overweight and to 26.8% (RR 2.72, 95% CI 2.04-3.64, P<0.01) and 32.3% (RR 2.82, 95% CI 2.27-3.50, P<0.01) among obese children. There was a trend of association of prehypertension with the time spent watching Television, internet and electronic games. Children whose mother or father had a history of hypertension had a trend to be prehypertensive or hypertensive. A parental hypertension was found in 33.6% of normotensive, 38.2% of prehypertensive, and 42.6% of hypertensive children (P=0.05). Children with prehypertension or hypertension were more likely to have a diabetic father or mother (22.8% and 22.6% vs 15.8%, respectively, P=0.01). Also, prehypertension and hypertension were associated with shorter gestational age, early birth, reduced birth weight, and shorter breastfeeding. CONCLUSION: Prehypertension and hypertension have a high prevalence among children in North Africa. They are associated with overweight, obesity, diabetes, a shorter gestational age, a lower birth weight and a shorter breastfeeding.

Association between MDR1 gene polymorphisms and the risk of Crohn's disease in a cohort of Algerian pediatric patients.

BACKGROUND: The multi-drug resistance gene (MDR1) has raised increasing interest as a susceptibility gene for Crohn's disease (CD). The role of MDR1 single-nucleotide polymorphisms (SNPs) in the predisposition and behavior of CD in the pediatric population is still elusive. Here, we investigated whether SNPs in MDR1 are associated with CD in Algerian pediatric patients. METHODS: A case-control study was conducted enrolling 47 pediatric CD patients and 100 controls. All subjects were genotyped for the most common MDR1 SNPs (C3434T, C1236T, and G2677A/T) using PCR-RFLP method. We also explored the association between polymorphisms and clinical sub-phenotypes. RESULTS: We have detected no significant association of C3435T SNP and pediatric CD. However, we observed a significantly higher frequency of the risk alleles, 1236T and 2677T/A among the CD patients compared to controls. Moreover, the risk allele 1236T was associated to a higher risk for resective surgery. CONCLUSION: Our data suggest that the C1236T and G2677A/T SNPs in the MDR1 gene are associated with CD and the C1236T risk allele with a more severe course of disease in Algerian pediatric patients. Further analysis using larger patients group and functional studies would be interesting to elucidate the role of MDR1 gene in pediatric CD.Pediatric Research (2016); doi:10.1038/pr.2016.163.