Disability and survival in Duchenne muscular dystrophy
Disability and survival in Duchenne muscular dystrophy
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DOI：
10.1136/jnnp.2007.141721
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发表时间：
2009-03-01
影响因子：
11
通讯作者：
Bloch, K. E.
中科院分区：
文献类型：
--
作者：
Kohler, M.;Clarenbach, C. F.;Bloch, K. E.
背景：杜氏肌营养不良症（DMD）导致肌肉功能进行性损害、呼吸衰竭和过早死亡。关于身体残疾和呼吸功能过程的纵向数据很少。目的：前瞻性评估DMD患者的身体损伤和残疾、呼吸功能和生存率，以描述当前护理下的病程。方法：对43例年龄在5-35岁的DMD患者，采用杜氏肌营养不良物理损伤和护理依赖（DID）评分进行年度身体残疾评估，评分范围从9分（无残疾）到80分（完全依赖），以及用力肺活量（FVC），平均时间间隔为5.4年（SD 2.1）。结果：DID评分与年龄呈双曲函数关系(f = 85.36age/(10.05+age), R = 0.62, p = 0.62
Background: Duchenne muscular dystrophy (DMD) leads to progressive impairment of muscle function, respiratory failure and premature death. Longitudinal data on the course of physical disability and respiratory function are sparse.Objectives: To assess prospectively physical impairment and disability, respiratory function and survival in patients with DMD over several years to describe the course of the disease with current care.Methods: In 43 patients with DMD, aged 5-35 years, yearly assessments of physical disability by the Duchenne muscular dystrophy physical Impairment and Dependence on care (DID) score, ranging from 9 (no disability) to 80 (complete dependence), and forced vital capacity (FVC), were obtained over a mean time interval of 5.4 (SD 2.1) years.Results: DID scores were correlated with age according to a hyperbolic function (f = 85.36age/(10.05+age), R = 0.62, p