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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Sleep disturbance is a common health problem that can influence the quality of life. There are several types of sleep disorders, such as obstructive sleep apnea, insomnia, narcolepsy, periodic limb movement disorder, and circadian dysregulation. Medical students are probably more prone to sleep disturbances due to their extreme academic stress. In this research, the incidence of sleep disturbance among medical students was examined, and the concomitant risk factors were determined. That was one-time research. A questioning was used to collect social, demographic and sleeping data. 678 1st, 2nd and 3rd year medical students were surveyed. 29% complained of at least one sleep disturbance. The most widely spread sleep disturbance observed among 51.8% medical students included insomnia (initial insomnia and sleep maintenance). 4th year students and those who spend much time on smartphones were more prone to sleep disturbances. Sleep disturbances are common among medical students. They need to be discovered and paid attention to before the situation gets worse.

Social factors such as bad living conditions, harmful labor conditions, low quality of medicine, loss of social contacts, etc., and lifestyle factors such as mal- and over-nutrition, hypodynamia, bad habits, etc. are the main issues of premature ageing. In this article, the alimentary factor such as the risk of premature ageing has been considered from the point of view of over- and malnutrition (protein-e nergy undernutrition) and its influence on the rate of ageing. The effect of overnutrition has been examined in the literature review of Russian and foreign investigators. Scientific publications show that one alimentary factor can be used to produce a significant influence on life span and frequency of some degenerative diseases. The relationship between malnutrition in the older population and biological age has been evaluated. 408 those surveyed from the city of Voronezh aged 55 to 70 were included into the study. The examination was nonrecurrent. The biological age of those surveyed was determined using the method by V. P. Voitenko. Malnutrition was assessed with the MNA (Mini Nutritional Assessment) tool. Malnutrition is observed among 2.2% of elderly patients, 58.8% of those surveyed are at risk for malnutrition, whereas 39% of them have a normal alimentary status. It is established that the biological age is correlated with the alimentary status. Statistically significant differences are found in the mean group values of the biological age in the subgroup of people with a normal alimentary status in relation to the subgroup of people with a risk for malnutrition (p<0.05): the values were 56±1.7 and 64±1.4, respectively. The publication shows a direct and indirect influence of the alimentary status (both overnutrition, and malnutrition) on the rate of senescence.