Analysis of the incidence of primary immunodeficiency disorders in the Samara region. Clinical cases

Research objective. This research was conducted to identify and to evaluate clinical and anamnestic features of primary immunodeficiency disorders (PID) as well as quantitive and qualitive microflora composition in PID-patients in Samara region. Methods. We analysed 29 case histories of patients with PID aged from 3 to 55 years old (20 men, 9 women). Clinical and anamnestic features of the diseases were studied. We also examined microflora of 34 loci from upper respiratory tract of 7 patients. Taking into account a small sample we used statistical analysis of variance by criterion F, the normality of the distribution was evaluated by the Shapiro-Wilk criterion (S-U), the distribution was considered normal when S-U value was <0,05. Results. Humoral immune deficiency takes the first place in the structure of PID (62,1%). In 67% the PID started with infections of bronchopulmonary system. Initial manifestations of PID appeared already in childhood (1,65±0,97 y.o.), the average age of establishing the diagnosis was 11,1±9,62 years (the difference was 9,6 years; p<0,001). The infectious syndrome took place further in all cases. The most frequent manifestations were respiratory system diseases (92%). We examined respiratory tract mucose membrane microbiota in 7 patients: all patients, except the woman with common variable immunodeficiency (CVID), had dysbiosis of respiratory tract mucose membrane characterized by clinically relevant microflora in unusual loci and decreased normobiota’s titers. Conclusion. PID are quite rare conditions. Nowadays 29 patients with established diagnosis of PID are registered in Samara region. This reflects the low prevalence of the diagnosed PID; at the same time the detection of the disease in adult age reflects the low level of diagnostics. More severe disorders were revealed in patients with late disease detection. The PID in clinical case 1 was diagnosed at the age of 16 y.o., and in clinical case 2 - only at the age of 40 y.o. The diagnostics of mycoses, virus infections or mycobacteriosis in this group of patients might be very important.

primary immunodeficiency disorders, innate immunity system, humoral immune deficiency, cellular immune deficiency, immunopathological syndroms, microbiota of upper respiratory tract