HISTORICAL AND MODERN ASPECTS OF CYSTIC FIBROSIS IN RUSSIA

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.