OBJECTIVES : To analyze the specialized medical care provision for children with vital and chronic progressed rare (orphan) diseases associated with life expectancy decrease and disability (vital rare diseases) in Russian Federation.

METHODS : Analysis of actual legal acts for specialized and high-technology medical care provision was done as well as data from 72 Russian regions concern specialized and high-technology medical care provision for vital rare diseases were gathered.

RESULTS : The number of children from regional segments of Federal registry of individuals suffered from vital rare diseases that were provided with specialized and high-technology medical care were established. The most frequently appeared diseases with such medical care requests were detected. Situation with specialized and high-technology medical care provision was analyzed in different federal areas. Mostly of children with vital rare diseases were not provided with specialized and high-technology medical care during the whole year. Such situation can lead to late correction of therapy in terms of diseases progression, disability increase, quality and duration of life decrease, higher costs of treatment.

CONCLUSIONS : Patients’ route system creation in terms of diseases features and Russian regions possibilities is one of the most important area of necessary medical care improvement for such categories of patients. Pharmacoeconomic study will also be an argument for relevant budget allocation on state level concerns specialized medical care provision for vital rare diseases.