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肌萎缩侧索硬化症诊断时的肺功能。恶化率。

Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration.

作者信息

Schiffman P L, Belsh J M

机构信息

Department of Medicine, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, New Brunswick 08903-0019.

出版信息

Chest. 1993 Feb;103(2):508-13. doi: 10.1378/chest.103.2.508.

DOI:10.1378/chest.103.2.508
PMID:8432145
Abstract

The purpose of this study was to determine the degree of respiratory muscle impairment in patients with newly diagnosed amyotrophic lateral sclerosis (ALS) and the subsequent rate of decline of respiratory function. Thirty-one of 36 patients had respiratory muscle weakness at presentation, although only 7 complained of any respiratory symptoms. Vital capacity (percent predicted) was significantly lower in the symptomatic group (55.9 +/- 20.3) compared with the asymptomatic group (76.4 +/- 21.0). Respiratory muscle impairment as measured by vital capacity (percent predicted) was related to stage of disease at presentation. Rate of decline of respiratory muscle strength as measured by VC (-3.5 percent/month), negative inspiratory pressure (NIF) (+2.9 cm H2O/month), and positive expiratory pressure (PEP) (-3.4 cm H2O/month) tended to be linear with a great deal of interpatient variability. It is concluded that early measurement of respiratory muscle strength in ALS with subsequent follow-up studies may be useful in determining overall prognosis and in decision making.

摘要

本研究的目的是确定新诊断的肌萎缩侧索硬化症(ALS)患者呼吸肌受损的程度以及随后呼吸功能的下降速率。36例患者中有31例在就诊时存在呼吸肌无力,尽管只有7例主诉有任何呼吸症状。有症状组的肺活量(预测值百分比)(55.9±20.3)显著低于无症状组(76.4±21.0)。以肺活量(预测值百分比)衡量的呼吸肌受损与就诊时的疾病阶段相关。以肺活量(VC)(-3.5%/月)、吸气负压(NIF)(+2.9 cm H₂O/月)和呼气正压(PEP)(-3.4 cm H₂O/月)衡量的呼吸肌力量下降速率往往呈线性,患者间存在很大差异。得出的结论是,对ALS患者进行呼吸肌力量的早期测量并随后进行随访研究,可能有助于确定总体预后和决策。

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