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.

The evolution of digital technologies leads to a tectonic transformation of all spheres of society. Law, as a system of regulating public relations, is changing dynamically along with the development of public relations in different spheres. The development of IT led to the emergence of blockchain technology, which, in turn, became the basis for the development of smart contracts. Smart contract technology, as it develops, causes changes not only in the legislation, but also in the model of interaction between the state and business. Due to smart contracts, a significant part of the rules can be algorithmized, and the regulation can become machine-readable. Purpose of the research: Legal research of the current legislation, the synergy of business, law and economy in the implementation of smart contract technology, determination of theoretical concepts in relation to smart contracts, the content and problems of the application of smart contracts, and identification of the most significant proposals for improving legislation. Methods: The authors of the research used general and specific scientific methods. In the study of the technological foundations of the smart contract, the main methods were analysis, synthesis, analogy, and a system-structural approach.

Currently, entrepreneurial and professional activities are being transformed towards digital business, management, economics and technology, which are viewed through the prism of four new technological areas: artificial intelligence, blockchain, cloud technologies and data analytics. Therefore, we will use multidisciplinary approaches that show how digital tools affect all areas of business processes, management and economics. Purpose of the study: to determine the role and importance of artificial intelligence for building an effective model of business, management, economy and technology transformation, business and professional activities in the context of law and society digital transformation. Methods: The methodological basis of this work was general scientific methods of cognition of legal phenomena, such as synthesis, the method of analogy, formal logic and others, as well as specific scientific methods for studying the category of artificial intelligence in the field of entrepreneurial and professional activity. Results and novelty: To achieve the goals of using AI, it can be described as follows: the first step is to use big data, the second is to apply analytics, and the third is forecasting. AI is used to collect and store data for analysis and further forecasting. Companies in IT, marketing, finance, accounting and sales are using AI to become more competitive and efficient.

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The problem of transition in the line of business assets and obligations succession with regard to norms of civil, family and business law represents an important legal problem as for the matter of family business succession. Russian legislation does not determine the family business succession as a single entity, there exists no special regulation as well as the term “entrepreneurial succession”. The doctrine gives a reasonable conclusion that “practices of the recent past reveal substantial problems of marital regimes legal regulation under a digital transformation of the economy”. Inheriting different properties that can be collectively referred to sphere of entrepreneurial activity causes many problems of similar properties transition in the line of succession in the field of law enforcement. Determining particularities of legal regulation of inheritance relations complicated with business activities in order to ensure efficient regulation of succession to business assets and debts and as well to ensure law enforcement stability. The methodological base for the present scientific research is represented by the system of general scientific and specific scientific methods and research techniques, including a historical method, a logical method, a method of system analysis and research, a comparative legal method, a statistical method, a functional-structural method, methods of analysis and synthesis, a method of specification, an empirical and theoretical method, i.e. analogy, deduction. The authors suppose that in conditions of the world financial crisis complicated with consequences of the coronavirus pandemic small businesses are the most vulnerable, including family businesses. The authors believe that a modern lawyer must have systemic knowledge for efficient application of civil law, inheritance law, family law, entrepreneurial law on the basis of the convergence principle in law. The use of a rather broad methodological base allows determining essential properties of legal regulation of the family members’ entrepreneurial activity and inheritance relations from the point of view of law enforcement problems resolution. As for particularities of inheritance regulations application, a joint-stock company is supposed to have certain mechanisms of the protection of its interests in terms of its shares inheritance. For example, it is possible to envisage the right of a private joint-stock company to discourage inclusion within its shareholders a new participant in line with a similar power of the limited liability companies.