REMEDIUMREMEDIUM1561-59362658-3534Joint-Stock Company Chicot140710.32687/1561-5936-2022-26-1-82-87Original ArticleRare diseases in the subjects of the Russian Federation: are these patients rare?KomarovI. A.iliya_komarov@mail.ruKrasilnikovaE. Yu.-AleksandrovaO. Yu.-N. A. Semashko National Research Institute of Public Health15122022261828729032022Copyright © 2022,2022This article presents data from the regional segments of the Federal Register of Persons Suffering from Life-threatening and Chronic Progressive Rare (Orphan) Diseases that Reduce Life Expectancy or Disability of Citizens, on the number of patients in the subjects of the Russian Federation with these diseases per 100,000 population in the Russian Federation at the end of 2020. We also analyzed the data of the regional segments of the Federal Register on the number of patients with the most expensive diseases from those presented in the leading regions in terms of the number of patients included in the regional segment of the Federal Register. At the same time, the data of the regional segments of the Federal Register on the number of patients with the most expensive diseases in the subjects with the smallest number of such patients are presented. Based on the analysis, the subjects of the Russian Federation were identified, in which the level of provision of patients with rare life-threatening diseases with drugs in 2020 was 65% and above. Also in this article are the subjects of the Russian Federation, in which the level of provision of patients with rare life-threatening diseases with drugs in 2020 was 50% or lower. Finally, the availability of medicines for patients with certain rare life-threatening diseases was established as a proportion of patients receiving medicines from the number included in the corresponding register.rare diseasesorphan drugsdrug supplyhealthcarefinancingfederalizationbudgetregions of the Russian Federationздравоохранениередкие болезнилекарственное обеспечениеорфанные лекарственные препаратыбюджетрегионы РФ1.Соколов А. А., Волгина С. Я., Нагибин О. А. Анализ оказания специализированной медицинской помощи пациентам с редкими заболеваниями // Проблемы стандартизации в здравоохранении. 2019. № 7-8. С. 51-62.2.Соколов А. А., Александрова О. Ю., Комаров И. А. Проблемы оказания высокотехнологичной медицинской помощи пациентам, страдающим редкими заболеваниями // Проблемы стандартизации в здравоохранении. 2019. № 7-8. С. 63-72.3.Ежегодный бюллетень Экспертного совета по редким (орфанным) заболеваниям. М., 2021. 244 с.4.Taruscio D., Vittozzi L., Stefanov R. National plans and strategies on rare diseases in Europe // Advances in Experimental Medicine and Biology. 2010. № 686. P. 475-491.5.Miller K. L., Lanthier M. Investigating the landscape of US orphan product approvals // Orphanet Journal of Rare Diseases. 2018. № 13:183. Р. 1-8.