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夏科-马里-图思综合征的康复管理:文献系统综述

Rehabilitation Management of the Charcot-Marie-Tooth Syndrome: A Systematic Review of the Literature.

作者信息

Corrado Bruno, Ciardi Gianluca, Bargigli Chiara

机构信息

From the Department of Public Health, University of Naples Federico II (BC, GC, CB), Naples, Italy.

出版信息

Medicine (Baltimore). 2016 Apr;95(17):e3278. doi: 10.1097/MD.0000000000003278.

DOI:10.1097/MD.0000000000003278
PMID:27124017
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4998680/
Abstract

The Charcot-Marie-Tooth disease (CMT) causes significant muscular deficits in the affected patients, restricts daily activities (ADL), and involves a severe disability. Although the conservative intervention is the only treatment for the disease, there is no scientific evidence so far on rehabilitation treatment. Objectives of the review are: research the best literary evidence so far on the rehabilitation treatment of CMT; critically analyze the outcome, to build an evidence-based work protocol.A systematic review of the rehabilitation of a patient with CMT, including the results from the following databases: Pubmed, Medline, Embase, Pedro, Cinahl, Ebsco discovery. Criteria for inclusion: randomized/controlled studies, analytic studies, transversal studies on a cohort of at least 10 individuals; medium/long-term report of the results.Eleven studies in total have been admitted to the final review phase; trials about physiotherapy CMT treatment (5), about orthosis treatment (6). Despite the wide range of outcomes and proposed interventions, the data points to the following: strength or endurance trainings improve functionality and ADLs of affected patients, while orthotic role is, at the moment, not completely clear.Physiotherapy treatment is a useful tool to manage CMT; more studies on a larger number of cases are needed to define orthosis utility and to establish the gold standard of the treatment.

摘要

夏科-马里-图思病(CMT)会给患病患者造成严重的肌肉功能缺陷,限制日常活动(ADL),并导致严重残疾。尽管保守干预是该病唯一的治疗方法,但目前尚无关于康复治疗的科学证据。本综述的目的是:研究目前关于CMT康复治疗的最佳文献证据;批判性地分析结果,以制定基于证据的工作方案。对一名CMT患者的康复情况进行系统综述,包括以下数据库的结果:PubMed、Medline、Embase、Pedro、Cinahl、Ebsco discovery。纳入标准:随机/对照研究、分析性研究、对至少10名个体的队列进行的横向研究;结果的中长期报告。共有11项研究进入最终综述阶段;关于CMT物理治疗的试验(5项),关于矫形器治疗的试验(6项)。尽管结果和建议的干预措施范围广泛,但数据表明:力量或耐力训练可改善患病患者的功能和ADL,而目前矫形器的作用尚不完全清楚。物理治疗是管理CMT的一种有用工具;需要对更多病例进行更多研究,以确定矫形器的效用并建立治疗的金标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ada/4998680/0097f2f47fef/medi-95-e3278-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ada/4998680/0097f2f47fef/medi-95-e3278-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ada/4998680/0097f2f47fef/medi-95-e3278-g002.jpg

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J Peripher Nerv Syst. 2014 Dec;19(4):328-32. doi: 10.1111/jns.12100.
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Hereditary motor and sensory neuropathies: Understanding molecular pathogenesis could lead to future treatment strategies.遗传性运动和感觉神经病:了解分子发病机制可能会带来未来的治疗策略。
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