Neuromuscular DiseasesNeuromuscular DiseasesНервно-мышечные болезни2222-87212413-0443Publishing House ABV Press2510.17650/2222-8721-2014-0-3-13-19LECTURES AND REVIEWSЛЕКЦИИ И ОБЗОРЫAdequate managment of patients with dystrophinopathies (muscular dystrophy Duchenne/Becker): objective scales and additional diagnostic methodsАдекватный менеджмент пациентов с дистрофинопатиями (мышечная дистрофия Дюшенна/Беккера): применение объективизирующих шкал и дополнительных методов исследованияNoskoA. S.НоскоА. С.
Russian Federation
nosko.anastasia@yandex.ruKurenkovA. L.КуренковА. Л.
Russian Federation
NikitinS. S.НикитинС. С.
Russian Federation
ZykovV. P.ЗыковВ. П.
Russian Federation
ROS “Specialists on Neuromuscular Disorders Society”, MoscowГБОУ ДПО РМАПО Минздрава России, МоскваSBEI RMAPGS Ministry of Public Health Russia, MoscowФГБНУ «Научный центр здоровья детей», МоскваFSBSI “Sientific Centre of Children Health”, MoscowРегиональная общественная организация «Общество специалистов по нервно-мышечным болезням», Москва190920144313191902201519022015Copyright ©; 2014, Nosko A.S., Kurenkov A.L., Nikitin S.S., Zykov V.P.Copyright ©; 2014, Носко А.С., Куренков А.Л., Никитин С.С., Зыков В.П.2014Nosko A.S., Kurenkov A.L., Nikitin S.S., Zykov V.P.Носко А.С., Куренков А.Л., Никитин С.С., Зыков В.П.https://creativecommons.org/licenses/by/4.0https://nmb.abvpress.ru/jour/article/view/25

There are still no guidlines on managment of Duchenne/Becker myodystrophy in domestic medical practice. It leads to decrease of quality of life and, what is more important, lifespan of patients. In this article we have described our Western coleagues lаst decade experience, including consensus guidelines published in 2010 on mаnаgment of Duchenne myodystrophy, supplemented with our practicle experience. We have described standardized motor development scale and muscle tone score for patients with MDD/MDB, and algorithm of multidiscipline care with focus on prevention, diagnosis and treatment of main disease and steroid therapy complications: cardiovascular, orthopedics, respirator etc. These recommendations not only improve quality of live and extend lifespan of MDD/MDB patients, but allow to take part in multicentre trials on searching of pathognomonic and symptomatic treatment.

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