Congenital defects in the immune system in the Stavropol region

Abstract

The study analyzed 88 patients with congenital immunodeficiencies registered in the Stavropol Region (SR) during a 10year period (2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018)(2019). The results established that the prevalence of primary immunodeficiencies (PID) in the SR was 3.1 per 100,000 population. Children affected by PID predominated (74.7 %) in the cohort of living patients. Twenty-seven nosological forms of PID were verified in the SR register. The most common was defective humoral component of the immune system (44.3 %), followed by combined immune disorders with syndromic manifestations (15.9 %) and phagocytosis defects (10.2 %). The molecular genetic diagnosis was confirmed in 38.6 % of patients. Hematopoietic cell transplantation was performed in 11.4 % of patients. Intravenous immunoglobulin medications were administered in 20.2 % of patients. Mortality in the PID cohort within the SR register was 6.9 %.