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杜氏肌营养不良症和脊髓性肌萎缩症中机械通气需求的指标

Indicators of need for mechanical ventilation in Duchenne muscular dystrophy and spinal muscular atrophy.

作者信息

Lyager S, Steffensen B, Juhl B

机构信息

Institute of Physiology, University of Aarhus, Denmark.

出版信息

Chest. 1995 Sep;108(3):779-85. doi: 10.1378/chest.108.3.779.

DOI:10.1378/chest.108.3.779
PMID:7656633
Abstract

STUDY OBJECTIVES

The purpose was to investigate a possible relationship between different parameters of physical function, spirometric measurements, and the approaching need for mechanical ventilation.

DESIGN

A nonrandomized, prospective, descriptive study of 11 patients with spinal muscular atrophy type II (SMA-II) and 14 patients with Duchenne muscular dystrophy (DMD). At a home visit, the anthropometric indices of age, height, and weight were recorded, the degree of disability was scored, and measurement of the strength of eight muscle groups and spirometry was performed. The interdependence of the variables was analyzed and the intergroup differences evaluated. Eighteen months later, it was found that one of the authors (B.J.), who was blind to the results of the first examination had instituted home mechanical ventilation on seven of the patients. The data were analyzed retrospectively for their predictive value as indicators of approaching ventilator dependency.

RESULTS

The seven patients who needed mechanical ventilation were the patients with DMD with the highest disability score (Egen Klassifikation [EK] sum > 20) and the smallest values for FVC < 1.2 L (FVC% < 30). We found a significant correlation (p = 0.002) between FVC% and the EK sum at the first examination and between the FVC% and the time until treatment with mechanical ventilation was instituted (p = 0.023). Although 7 of the 11 patients with SMA type II had FVC below 1.2 L and some of them had an EK sum score higher (indicating more disability) than some patients with DMD who needed mechanical ventilation, none of them required mechanical ventilation.

CONCLUSION

In this investigation, a combination of EK sum and FVC% provided a better indication of the approaching need for mechanical ventilation in the patients with DMD than the variables separately.

摘要

研究目的

本研究旨在探究身体功能的不同参数、肺功能测量结果与即将需要机械通气之间的可能关系。

设计

对11例II型脊髓性肌萎缩症(SMA-II)患者和14例杜氏肌营养不良症(DMD)患者进行非随机、前瞻性描述性研究。在一次家访中,记录年龄、身高和体重等人体测量指标,对残疾程度进行评分,并对八组肌肉力量进行测量以及进行肺功能测定。分析变量之间的相互依存关系并评估组间差异。18个月后,发现其中一位对首次检查结果不知情的作者(B.J.)已对7例患者实施了家庭机械通气。对数据进行回顾性分析,以评估其作为即将出现通气依赖指标的预测价值。

结果

需要机械通气的7例患者是残疾评分最高(埃根分类法[EK]总和>20)且用力肺活量(FVC)最小(FVC<1.2L,FVC%<30)的DMD患者。我们发现首次检查时FVC%与EK总和之间存在显著相关性(p=0.002),FVC%与开始进行机械通气治疗的时间之间也存在显著相关性(p=0.023)。虽然11例II型SMA患者中有7例FVC低于1.2L,其中一些患者的EK总和评分高于一些需要机械通气的DMD患者(表明残疾程度更高),但他们中没有一人需要机械通气。

结论

在本研究中,与单独的变量相比,EK总和与FVC%相结合能更好地提示DMD患者即将需要机械通气。

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