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伴有膈肌功能障碍的神经性肌萎缩患者肺功能恢复的模型构建

Modeling of Lung Function Recovery in Neuralgic Amyotrophy With Diaphragm Impairment.

作者信息

Rice Brenda L, Ashton Rendell W, Wang Xiao-Feng, Shook Steven J, Mireles-Cabodevila Eduardo, Aboussouan Loutfi S

机构信息

Respiratory Institute, Department of Pulmonary, Allergy, and Critical Care Medicine.

Department of Quantitative Health Sciences.

出版信息

Respir Care. 2017 Oct;62(10):1269-1276. doi: 10.4187/respcare.05568. Epub 2017 Jul 11.

Abstract

BACKGROUND

Neuralgic amyotrophy is an inflammatory peripheral nerve disorder in which phrenic nerve involvement can lead to diaphragm paralysis. The prevalence, magnitude, and time course of diaphragm recovery are uncertain.

METHODS

This study modeled the course of recovery of lung function in 16 subjects with diaphragm impairment from neuralgic amyotrophy. The first and last available vital capacity, sitting-to-supine decline in vital capacity, and maximal inspiratory pressures were compared.

RESULTS

An asymptotic regression model analysis in 11 subjects with at least partial recovery provided estimates of the vital capacity at onset (47%, 95% CI 25-68%), the final vital capacity (81%, 95% CI 62-101%), and the half-time to recovery (22 months, 95% CI 15-43 months). In those subjects, there was a significant improvement between the first and last measured FVC (median 44-66%, = .004) and maximal inspiratory pressure (mean 34-51%, = .004). Five subjects (31%) with complete recovery had a final sitting-to-supine drop of vital capacity of 16% and a maximal predicted inspiratory pressure of 63%.

CONCLUSIONS

Sixty-nine percent of subjects with diaphragm impairment from neuralgic amyotrophy experience recovery of lung function and diaphragm strength, but recovery is slow and may be incomplete.

摘要

背景

神经性肌萎缩是一种炎症性周围神经疾病,其中膈神经受累可导致膈肌麻痹。膈肌恢复的患病率、程度和时间进程尚不确定。

方法

本研究模拟了16例因神经性肌萎缩导致膈肌受损的受试者肺功能的恢复过程。比较了首次和最后一次可用的肺活量、从坐位到仰卧位肺活量的下降以及最大吸气压力。

结果

对11例至少部分恢复的受试者进行的渐近回归模型分析提供了发病时肺活量的估计值(47%,95%可信区间25 - 68%)、最终肺活量(81%,95%可信区间62 - 101%)以及恢复的半衰期(22个月,95%可信区间15 - 43个月)。在这些受试者中,首次和最后一次测量的用力肺活量(中位数44 - 66%,P = .004)和最大吸气压力(平均34 - 51%,P = .004)之间有显著改善。5例(31%)完全恢复的受试者从坐位到仰卧位肺活量的最终下降为16%,最大预测吸气压力为63%。

结论

69%因神经性肌萎缩导致膈肌受损的受试者经历了肺功能和膈肌力量的恢复,但恢复缓慢且可能不完全。

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