Treatment for Patients with Primary Immunodeficiency Diseases

Abstract

Primary immunodeficiency diseases occur with a frequency of 1: 10,000 pp. among population. The purpose of this study is to evaluate the most common forms of primary immunodeficiency among patients in Moscow based on the Russian registry for primary immunodeficiency, as well as to analyse the treatment tactics of three patients. The studies were conducted in 2016-2018 among 73 patients aged 5-12 from Russian Children's Clinical Hospital (RCCH), all diagnosed with primary immunodeficiency. The classification of patients was carried out according to the Russian registry by type of primary immunodeficiency. Lethal cases, gender and age distribution were considered. The treatment tactics of three patients were analysed. The number of boys exceeds the number of girls by 7.2 times (p ≤ 0.001) for defects in antibody production, by 6.0 times (p ≤ 0.001) for combined immunodeficiency, by 1.4 times (p ≤ 0.05) for phagocytic defects, and by 2.5 times (p ≤ 0.01) for other types of immunodeficiency. The patients with defects in antibody production predominated, namely by 1.5 times - patients with combined immunodeficiency (p ≤ 0.05), by 2.8 times - patients with phagocyte defects (p ≤ 0.01), by 4.7 times - patients with other types of immunodeficiency (p ≤ 0.001). 73 patient patients under the age of 12 years with primary immunodeficiency had their anamnesis analysed that helped to identify 4 main forms. Boys prevailed in gender composition (80.8%), and there were no significant differences between boys and girls in age. The lethality prevailed among patients with combined type immunodeficiency. Thus, there were 1.3 times less lethal cases among patients with phagocyte defects comparing to the combined type (p ≤ 0.05), but 2.4 times more compering to the other types of immunodeficiency (p ≤ 0.01).

Keywords: Primary immunodeficiency diseases, Patient registry, Combined immunodeficiency, Defects in antibody production.