Bulletin of Semashko National Research Institute of Public HealthBulletin of Semashko National Research Institute of Public Health2415-84102415-8429FSSBI «N.A. Semashko National Research Institute of Public Health»274110.25742/NRIPH.2024.02.001Обзорная статьяSystematic review of primary immunodeficiencyPakhomovaYana A.yanapakhomova@mail.ruSavvina2Nadezhda V.nadvsavvina@mail.ruhttps://orcid.org/0000-0003-2441-6193Medical institute of M.K.Ammosov North-Eastern Federal UniversityRepublican Clinical Hospital No. 304062024251029042025Copyright © 2025,2025<p>Primary immunodeficiency (PID) conditions are a heterogeneous group of diseases manifesting under the guise of various clinical manifestations. The prevalence of PID is higher in the group of patients under 18 years of age, but it is also found in adulthood. The purpose of the publication was to search for publications on the epidemiology of PID, awareness of medical personnel about PID and immune reactions, as well as the impact of the disease on the quality of life of patients and their families. Literature search was carried out on the databases Scopus, Web of Science, MedLine, PubMed, CyberLeninka, E-library. Current trends in the management of patients with PID are focused on the application of a multidisciplinary approach. Due to the high prevalence of PID in pediatric practice, the quality of life of patients with PID and their families decreases, manifested in the restriction of physical activity and the possibility of free movement, travel, social difficulties and psychological problems. School-age patients have learning difficulties. 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