Open AccessEpilemiological characteristics of Diamond–Blackfen anemia in pediatric population of the Russian Federation
Author(s) -
В. М. Чернов,
Г. С. Овсянникова,
Н. Б. Юдина,
А. В. Рогов,
Н. Е. Соколова,
А. В. Шамин,
О. В. Сурьянинова,
И. И. Спичак,
Т. И. Бурлуцкая,
Л. А. Леандрова,
А. Н. Чижкова,
Т. Л. Костюрина,
И. М. Юнусова,
Ж. Х. Кумыкова,
М. В. Бурнацева,
Н. А. Попова,
А. П. Шапочник,
Е Г Бунина,
И. В. Пономарева,
Rinat Galiulin,
Л. А. Мазурок,
Tatyana Ksenzova,
И. И. Гущанская,
В. Н. Фокин,
В. Г. Стрелец,
Elena Volodicheva,
Е. Х. Цыденешеева,
Galiovichkova,
Н. С. Сметанина
Publication year - 2019
Publication title -
voprosy gematologii/onkologii i immunopatologii v pediatrii
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.108
H-Index - 3
eISSN - 2414-9314
pISSN - 1726-1708
DOI - 10.24287/1726-1708-2019-18-3-22-28
Subject(s) - russian federation , medicine , incidence (geometry) , epidemiology , population , pediatrics , demography , anemia , family medicine , environmental health , geography , physics , regional science , sociology , optics
Diamond–Blackfen Anemia (DBA) is a rare, clinically and genetically heterogeneous disorder from the group of congenital syndromes of bone marrow failure. The purpose of this work is to identify the main medical-frequency characteristics of DBA (incidence, prevalence, mortality, cartographic analysis) in children in the Russian Federation during the observation period 2011–2016. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The Russian register of DBA patients, which had been developed and maintained since 2012 year by NMITS DGOI, was used to obtained epidemiological data and their analysis. At the end of 2018 year 141 patients from 137 families were included in the register. The average annual incidence rate of children with DBA for the Russian Federation was 0.63 ± 0.034, the average annual prevalence rate – 5.75 ± 0.87 per 100 thousand newborns born alive; mortality rate – 2.12%; the cartographic method of research showed that the largest number of patients was registered in the Central Federal District of the country, which is explained by the high population living in this district.