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面肩肱型肌营养不良症的管理策略

Management strategies in facioscapulohumeral muscular dystrophy.

作者信息

Lu Junren, Yao Zhenjun, Yang Yi, Zhang Chi, Zhang Jian, Zhang Ying

机构信息

Department of Orthopedic Surgery, Zhongshan Hospital affiliated with Fudan University, Shanghai, China.

Department of Nursing, Zhongshan Hospital affiliated with Fudan University, Shanghai, China.

出版信息

Intractable Rare Dis Res. 2019 Feb;8(1):9-13. doi: 10.5582/irdr.2019.01016.

Abstract

Facioscapulohumeral muscular dystrophy (FSHD) also known as Landouzy-Dejerine disease, is an autosomal-dominant disorder of the skeletal muscles with the name according to the various muscle groups it affects: the face, shoulders and upper arms. It is the third most common genetic degenerative disorder of the skeletal muscles without specific patterns in all the affected individuals. At present there is no cure for the disease but numerous management strategies are available to improve the quality of life and prevent further degeneration of various muscle groups. This review aims to provide an insight on the management strategies for FSHD patients including both lifestyle and medical intervention.

摘要

面肩肱型肌营养不良症(FSHD),也称为兰杜齐 - 德热里纳病,是一种常染色体显性遗传性骨骼肌疾病,其命名依据是它所影响的不同肌肉群:面部、肩部和上臂。它是骨骼肌第三常见的遗传性退行性疾病,在所有受影响个体中没有特定模式。目前,这种疾病无法治愈,但有许多管理策略可用于改善生活质量并防止各个肌肉群进一步退化。本综述旨在深入探讨FSHD患者的管理策略,包括生活方式和医学干预。

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