Adequate managment of patients with dystrophinopathies (muscular dystrophy Duchenne/Becker): objective scales and additional diagnostic methods

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Abstract

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.

About the authors

A. S. Nosko

ROS “Specialists on Neuromuscular Disorders Society”, Moscow

Author for correspondence.
Email: nosko.anastasia@yandex.ru
Russian Federation

A. L. Kurenkov

SBEI RMAPGS Ministry of Public Health Russia, Moscow

Russian Federation

S. S. Nikitin

FSBSI “Sientific Centre of Children Health”, Moscow

Russian Federation

V. P. Zykov

ROS “Specialists on Neuromuscular Disorders Society”, Moscow

Russian Federation

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Copyright (c) 2014 Nosko A.S., Kurenkov A.L., Nikitin S.S., Zykov V.P.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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