Epilemiological characteristics of Diamond–Blackfen anemia in pediatric population of the Russian Federation

V. M. Chernov; Чернов В. М.; G. S. Ovsyannikova; Овсянникова Г. С.; M. B. Yudina; Юдина Н. Б.; A. V. Rogov; Рогов А. В.; N. E. Sokolova; Соколова Н. Е.; A. V. Shamin; Шамин А. В.; O. V. Suriyaninova; Сурьянинова О. В.; I. I. Spichak; Спичак И. И.; T. I. Burlutskaya; Бурлуцкая Т. И.; L. A. Leandrova; Леандрова Л. А.; A. N. Chizhkova; Чижкова А. Н.; T. L. Kostyurina; Костюрина Т. Л.; I. M. Yunusova; Юнусова И. М.; Zh. Kh. Kumikova; Кумыкова Ж. Х.; M. V. Burnatseva; Бурнацева М. В.; N. A. Popova; Попова Н. А.; A. P. Shapochnik; Шапочник А. П.; E. G. Bunina; Бунина Е. Г.; I. V. Ponomareva; Пономарева И. В.; R. Kh. Galiulin; Галиулин Р. Х.; L. A. Mazurok; Мазурок Л. А.; T. I. Ksenzova; Ксензова Т. И.; I. I. Gushchanskaya; Гущанская И. И.; V. N. Fokin; Фокин В. Н.; V. G. Strelets; Стрелец В. Г.; E. M. Volodicheva; Володичева Е. М.; E. Kh. Tsedenisheeva; Цыденешеева Е. Х.; G. A. Novichkova; Новичкова Г. А.; N. S. Smetanina; Сметанина Н. С.

doi:10.24287/1726-1708-2019-18-3-22-28

Epilemiological characteristics of Diamond–Blackfen anemia in pediatric population of the Russian Federation

Authors: Chernov V.M.¹^,2, Ovsyannikova G.S.¹, Yudina M.B.³, Rogov A.V.⁴, Sokolova N.E.⁵, Shamin A.V.⁶, Suriyaninova O.V.⁴, Spichak I.I.⁷, Burlutskaya T.I.⁸, Leandrova L.A.⁹, Chizhkova A.N.¹⁰, Kostyurina T.L.¹¹, Yunusova I.M.¹², Kumikova Z.K.¹³, Burnatseva M.V.¹³, Popova N.A.¹⁴, Shapochnik A.P.¹⁵, Bunina E.G.¹⁶, Ponomareva I.V.¹⁷, Galiulin R.K.¹⁸, Mazurok L.A.¹⁹, Ksenzova T.I.²⁰, Gushchanskaya I.I.²¹, Fokin V.N.²², Strelets V.G.²³, Volodicheva E.M.²⁴, Tsedenisheeva E.K.¹², Novichkova G.A.¹, Smetanina N.S.¹
Affiliations:
1. Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
2. N.I. Pirogov Russian National Research Medical University
3. Voronezh Regional Pediatric Clinic
4. Regional Pediatric Clinic
5. Pediatric City Hospital
6. Pediatric City Clinical Hospital
7. Chelyabinsk Regional Pediatric Clinic
8. Pediatric Regional Clinic
9. Lukashevsky A.S. Kamchatka Regional Hospital
10. KOMI Republic Hospital
11. Vologda Regional Pediatric Hospital
12. Pediatric Republic Clinic
13. Republic Pediatric Clinic
14. Volgograd Regional Oncological Center №1
15. Orenburg Regional Clinical Oncological Center
16. Kemerovo Regional Clinic №1
17. City Novosibirsk Oncological Clinic
18. Regional Clinic
19. Kurgan Regional Clinic
20. Regional Clinic №1
21. Bryansk Regional Hospital №1
22. Ivanovo Regional Clinic
23. Kostroma Regional Hospital
24. Tula Regional Clinic
Issue: Vol 18, No 3 (2019)
Pages: 22-28
Section: ORIGINAL ARTICLES
Submitted: 12.09.2019
Accepted: 12.09.2019
Published: 12.09.2019
URL: https://hemoncim.com/jour/article/view/256
DOI: https://doi.org/10.24287/1726-1708-2019-18-3-22-28
ID: 256

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Abstract

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.

Keywords

children, Diamond–Blackfan anemia, register, epidemiology, morbidity, prevalence, mortality, cartographic analysis

About the authors

V. M. Chernov

Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation;
N.I. Pirogov Russian National Research Medical University

Moscow Russian Federation

G. S. Ovsyannikova

Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

Author for correspondence.
Email: galina.ovsyannikova@fnkc.ru

Galina S. Ovsyannikova, MD, Hematologist at the Outpatient Department, Researcher in the Department of Hematology and Cell Technologies

117997, Moscow, Samory Mashela st., 1

Russian Federation

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