North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy

North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy
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DOI:
10.1016/j.nmd.2010.06.014
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发表时间:
2010-11-01
影响因子:
2.8
通讯作者:
Mercuri, Eugenio
Mercuri, Eugenio
中科院分区:
医学4区
文献类型:
--
作者:
Mazzone, Elena;Martinelli, Diego;Mercuri, Eugenio

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北极星动态评估是专门为患有Duchenne肌营养不良症(DMD)的流动男孩设计的功能量表。最近,6分钟步行测试也被用作DMD试验的结果衡量标准。我们研究的目的是使用北极星评估和6分钟步行测试来评估一大批受DMD影响的流动男孩。更具体地说,我们希望建立每个测量结果的光谱及其相关性。这是一项涉及10个中心的前瞻性多中心研究。队列包括112名流动的DMD男孩,年龄在4.10岁到17岁之间(平均8.18+/-2.3 DS)。112人中有91人服用类固醇:间歇性服用37/91,每日服药54/91。北极星评分从6/34到34/34不等。6min步行距离为127m~560.6 m,10m步行时间为3s~15s。S的起立时间为1~27.5分,部分患者无法起立。不出所料,结果随年龄增长而变化,接受每日激素治疗的患儿总体效果较好。北极星评分与6分钟步行试验和定时起地试验呈中等至良好的相关性,而与10m定时步行/跑试验的相关性较差。相比之下,6min步行试验与10m计时步行/跑试验的相关性好于与定时起跳的相关性。这些发现表明,这些结果测量的组合可以有效地用于流动的DMD男孩,并将提供有关运动功能的不同方面的信息,这些信息可能不是通过单一测量获得的。(C)2010年,爱思唯尔出版。
The North Star Ambulatory Assessment is a functional scale specifically designed for ambulant boys affected by Duchenne muscular dystrophy (DMD). Recently the 6-minute walk test has also been used as an outcome measure in trials in DMD.The aim of our study was to assess a large cohort of ambulant boys affected by DMD using both North Star Assessment and 6-minute walk test. More specifically, we wished to establish the spectrum of findings for each measure and their correlation.This is a prospective multicentric study involving 10 centers. The cohort included 112 ambulant DMD boys of age ranging between 4.10 and 17 years (mean 8.18 +/- 2.3 DS). Ninety-one of the 112 were on steroids: 37/91 on intermittent and 54/91 on daily regimen.The scores on the North Star assessment ranged from 6/34 to 34/34. The distance on the 6-minute walk test ranged from 127 to 560.6 m. The time to walk 10 m was between 3 and 15s. The time to rise from the floor ranged from 1 to 27.5 s. Some patients were unable to rise from the floor.As expected the results changed with age and were overall better in children treated with daily steroids.The North Star assessment had a moderate to good correlation with 6-minute walk test and with timed rising from floor but less with 10 m timed walk/run test. The 6-minute walk test in contrast had better correlation with 10 m timed walk/run test than with timed rising from floor. These findings suggest that a combination of these outcome measures can be effectively used in ambulant DMD boys and will provide information on different aspects of motor function, that may not be captured using a single measure. (C) 2010 Published by Elsevier B.V.