ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v10i6.896ppharm-157Research ArticleЛЕКЦИЯLECTUREИСТОРИЧЕСКИЕ И СОВРЕМЕННЫЕ АСПЕКТЫ МУКОВИСЦИДОЗА В РОССИИHISTORICAL AND MODERN ASPECTS OF CYSTIC FIBROSIS IN RUSSIAКапрановН. И.KapranovN. I.

доктор медицинских наук, заслуженный врач РФ, профессор Медико-генетического научного центра РАМН

PhD, Honored Doctor of Russia, Professor at the medical-genetic scientific center

nikolay.I.kapranov@gmail.comКаширскаяН. Ю.KashirskayaN. Y.АшероваИ. К.AsherovaI. K.КондратьеваЕ. И.KondratyevaE. I.ШерманВ. Д.ShermanV. D.Медико-генетический научный центр РАМН, МоскваРоссияMedical-genetic scientific center, MoscowRussian FederationМедико-генетический научный центр РАМН, Москва, Российская ФедерацияРоссияMedical-Genetic Scientific Center, Moscow, Russian FederationRussian FederationДетская клиническая больница № 1, Ярославль, Российская ФедерацияРоссияChildren’s Clinical Hospital № 1, Yaroslavl, Russian FederationRussian FederationМедико-генетический научный центр РАМН, Москва, Российская Федерация ДГКБ № 13 им. Н.Ф. Филатова, Москва, Российская ФедерацияРоссияMedical-Genetic Scientific Center, Moscow, Russian Federation Filatov CMCH № 13, Moscow, Russian FederationRussian Federation201302122013106Педиатрическая фармакология5360Copyright © Капранов Н.И., Каширская Н.Ю., Ашерова И.К., Кондратьева Е.И., Шерман В.Д., 20132013Капранов Н.И., Каширская Н.Ю., Ашерова И.К., Кондратьева Е.И., Шерман В.Д.Kapranov N.I., Kashirskaya N.Y., Asherova I.K., Kondratyeva E.I., Sherman V.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pedpharma.ru/jour/article/view/157

The article is dedicated to historical and modern trials in the sphere of mucoviscidosis in Russia, peculiarities of clinical manifestations, pathophysiological and pathomorphological disorders and of the disease course with the analysis of the first results of the register of patients in the Moscow region. The aggregate result of this evaluation is the survival rate in the Moscow region that has become almost equal to the one in the USA and Europe and is considerably higher than in the other regions of the Russian Federation. Such a result reflects many components of specialized care rendered to mucoviscidosis patients in the Moscow region: special centers for children and adults have been founded, patients are actively and regularly checked and may be examined in detail during each visit, while pharmacological support is up to world standards; this is not yet present or is developed insufficiently in many RF subjects. The article also dwells upon the genetic peculiarities of mucoviscidosis important both for diagnosing the disease (including neonatal screening) and creation of a panel for DNA-diagnostics and for determining the disease course to a considerable extent. It also presents information on the prospects of gene therapy development and the first results of clinical studies.

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The authors declare that there are no conflicts of interest present.