Introduction: Primary immunodeficiencies (PID) are a group of rare genetic disorders with a multitude of clinical symptoms. Characterization of epidemiological and clinical data via national registries has proven to be a valuable tool of studying these diseases. Materials and Methods: The Russian PID registry was set up in 2017, by the National Association of Experts in PID (NAEPID). It is a secure, internet-based database that includes detailed clinical, laboratory, and therapeutic data on PID patients of all ages. Results: The registry contained information on 2,728 patients (60% males, 40% females), from all Federal Districts of the Russian Federation. 1,851/2,728 (68%) were alive, 1,426/1,851 (77%) were children and 425/1,851 (23%) were adults. PID was diagnosed before the age of 18 in 2,192 patients (88%). Antibody defects (699; 26%) and syndromic PID (591; 22%) were the most common groups of PID. The minimum overall PID prevalence in the Russian population was 1.3:100,000 people; the estimated PID birth rate is 5.7 per 100,000 live births. The number of newly diagnosed patients per year increased dramatically, reaching the maximum of 331 patients in 2018. The overall mortality rate was 9.8%. Genetic testing has been performed in 1,740 patients and genetic defects were identified in 1,344 of them (77.2%). The median diagnostic delay was 2 years; this varied from 4 months to 11 years, depending on the PID category. The shortest time to diagnosis was noted in the combined PIDs-in WAS, DGS, and CGD. The longest delay was observed in AT, NBS, and in the most prevalent adult PID: HAE and CVID. Of the patients, 1,622 had symptomatic treatment information: 843 (52%) received IG treatment, mainly IVIG (96%), and 414 (25%) patients were treated with biological drugs. HSCT has been performed in 342/2,728 (16%) patients, of whom 67% are currently alive, 17% deceased, and 16% lost to follow-up. Three patients underwent gene therapy for WAS; all are currently alive. Conclusions: Here, we describe our first analysis of the epidemiological features of PID in Russia, allowing us to highlight the main challenges around PID diagnosis and treatment.

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Pneumococcal infections are among the most common infections are an important cause of morbidity and mortality worldwide, including Russia. At the end of 2014 in the national immunization schedule countries introduced vaccination against pneumococcal infection The aim of study. To assess the epidemiological effectiveness of vaccination against pneumococcal infection in young children selectively in different territories of the Russian Federation 2 years later from its beginning Materials and methods. In the course of epidemiological observational analytical research of type «case-control» data were obtained on the incidence in vaccinated and unvaccinated against pneumococcal infection in children from 2 months to 2 years We analyzed 1422 outpatients and 356 case histories of children in several cities (Moscow, Smolensk, Sevastopol, Perm, Tyumen, Barnaul). The results are subjected to statistical processing The results and discussion Established that there is a reliable causal connection between the lack of vaccination of children against pneumococcal infection and incidence of diseases. Shows high prophylactic efficacy of vaccination of children up to 2 years. Received in outpatient and inpatient facilities, evidence suggests that the incidence of invasive and non-invasive forms of pneumococcal infection was significantly lower in the vaccinated children It was revealed that the vaccination coverage against pneumococcal infection in children from 2 months to one year and the second year of life was 51 and 40% respectively. Vaccination in the first six months of life in 2016 received only 25% of children. A high proportion of the parent's refusal of vaccination of children (54.2%), and high frequency medical taps (9.7%) from vaccination.

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The incidence of acute intestinal infections in Russia is ranked 3rd 4th among all infectious diseases, and rotavirus is the most frequent etiologic factor in children under 5 years old. Almost 40% of children under the age of two have three episodes of rotavirus infection, the same group undergo the most severe course of the disease. Rotavirus gastroenteritis causes 197,000 annual deaths, 23 children die due to this infection every hour. The high resistance of rotavirus in the environment, the ease of transmission and the large number of asymptomatic carriers predetermine the need for timely vaccination. There are 2 licensed oral live attenuated rotavirus vaccines on the international market, one of which is RotaTeq pentavalent vaccine.