Inborn Errors of Immunity in Algerian Children and Adults: A Single-Center Experience Over a Period of 13 Years (2008-2021).

Background: Inborn errors of immunity (IEI) predispose patients to various infectious and non-infectious complications. Thanks to the development and expanding use of flow cytometry and increased awareness, the diagnostic rate of IEI has markedly increased in Algeria the last decade. Aim: This study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes. Methods: We collected and analyzed retrospectively the demographic data, clinical manifestations, immunologic, genetic data, and outcome of Algerian IEI patients - diagnosed in the department of medical immunology of Beni Messous university hospital center, Algiers, from 2008 to 2021. Results: Eight hundred and seven patients with IEI (482 males and 325 females) were enrolled, 9.7% of whom were adults. Consanguinity was reported in 50.3% of the cases and a positive family history in 32.34%. The medium age at disease onset was 8 months and at diagnosis was 36 months. The median delay in diagnosis was 16 months. Combined immunodeficiencies were the most frequent (33.8%), followed by antibody deficiencies (24.5%) and well-defined syndromes with immunodeficiency (24%). Among 287 patients tested for genetic disorders, 129 patients carried pathogenic mutations; 102 having biallelic variants mostly in a homozygous state (autosomal recessive disorders). The highest mortality rate was observed in patients with combined immunodeficiency (70.1%), especially in patients with severe combined immunodeficiency (SCID), Omenn syndrome, or Major Histocompatibility Complex (MHC) class II deficiency. Conclusion: The spectrum of IEI in Algeria is similar to that seen in most countries of the Middle East and North Africa (MENA) region, notably regarding the frequency of autosomal recessive and/or combined immunodeficiencies.

Shrimp sensitization in house dust mite algerian allergic patients: A single center experience.

Background: Cross-reactivity between shrimp and house dust mite (HDM) proteins has been widely documented. However, a significant geographical variability in sensitization patterns and cross-reactive allergens has been reported which may impact the diagnosis and management of shrimp allergy among HDM-shrimp co-sensitized patients. This study aimed to investigate the prevalence of shrimp and tropomyosin sensitization among HDM-allergic patients in order to understand the local epidemiology to inform the development of targeted diagnostic and therapeutic tools. Methods: Four hundred forty-six (446) HDM-allergic patients and 126 atopic controls were screened for shrimp-specific IgE using the IMMULITE 2000 XPI® System. HDM-shrimp sensitized subjected were also tested for IgE tropomyosin (nPen m 1) and thoroughly interviewed about their shellfish consumption habits. Tropomyosin sensitized patients were subjected to further analysis including measurement of IgE specific to squid and crab. Results: The prevalence of shrimp sensitization in the HDM-allergic population was 20.4% vs 0% in the control group. Of them 63.7% were clinically allergic to shrimp, while 9 cases had no history of allergic reaction to this food and 24 patients reported not having consumed shrimp before. Besides, 72.5% of the HDM-shrimp sensitized subjects had tropomyosin-specific IgE with a positivity rate of 82.8% among shrimp-allergic patients. Among tropomyosin reactors, 95.5% were sensitized to crab and 89.5% to squid, none of them had previously ingested neither crab nor squid. Nevertheless, one-third of HDM-shrimp sensitized patients who never consumed shrimp before did not react to tropomyosin. Conclusions: Shrimp allergy seems to be strictly dependent on HDM sensitization, at least in this geographical area. Therefore, HDM allergic patients should be systematically screened for shrimp sensitization and asked about the consumption of shellfish. Tropomyosin is a major and clinically relevant shrimp allergen that accounts for shellfish-HDM cross-reactivity. However, other components could be involved.