Effect of long-term steroids on cough efficiency and respiratory muscle strength in patients with Duchenne muscular dystrophy
Effect of long-term steroids on cough efficiency and respiratory muscle strength in patients with Duchenne muscular dystrophy
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DOI：
10.1542/peds.2006-1400
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发表时间：
2007-02-01
期刊：
影响因子：
8
通讯作者：
Amin, Raouf
中科院分区：
文献类型：
--
作者：
Daftary, Ameet S.;Crisanti, Mark;Amin, Raouf
目标。本研究的目的是确定长期类固醇治疗是否与杜氏肌营养不良患者咳流量峰值增加有关，并确定哪种肺功能测试变量最能预测咳流量峰值。在本病例对照研究中，我们检查了过去2年内在我院进行过肺功能检查的杜氏肌营养不良症患者的病历。类固醇治疗的患者治疗至少1年。测量的肺功能测试包括用力肺活量、最大呼气压、最大吸气压、最大自主通气和咳嗽流量峰值。采用多元线性回归分析确定哪种肺功能检查指标最能预测咳流量峰值，并评估类固醇治疗和患者年龄对咳流量峰值的影响。10例接受类固醇治疗的患者和25例未接受类固醇治疗的患者进行分析。类固醇治疗的患者咳嗽流量峰值和最大呼气压显著升高。每个肺功能测试变量与咳嗽流量峰值显著相关。调整后r(2)值最高的线性模型仅包括最大自主通气和类固醇治疗两个变量，表明类固醇治疗患者的咳嗽流量峰值比未治疗患者高27 L/min。最大自主通气与类固醇之间的相互作用无统计学意义，表明类固醇相关的咳嗽流量峰值增加在观察到的最大自主通气值范围内近似恒定。最大自主通气和治疗组对咳嗽峰值流的影响与患者年龄无关。长期类固醇治疗与杜氏肌营养不良患者咳嗽高峰流量和呼吸肌力量的改善有关。最大自主通气可能是杜氏肌营养不良患者肺功能的有用预测指标。
OBJECTIVE. The objective of this study was to determine whether long-term steroid therapy is associated with increased peak cough flow in patients with Duchenne muscular dystrophy and to determine which pulmonary function test variable is most predictive of peak cough flow.METHODS. In this case-control study, the medical charts of patients who had Duchenne muscular dystrophy and had pulmonary function tests at our institution in the previous 2 years were examined. Steroid-treated patients were on therapy for at least 1 year. The measured pulmonary function tests included forced vital capacity, maximum expiratory pressure, maximum inspiratory pressure, maximum voluntary ventilation, and peak cough flow. Multiple linear regression analysis was used to determine which pulmonary function test measure was most predictive of peak cough flow and assess the influence of steroid treatment and patient age on peak cough flow.RESULTS. Ten steroid-treated and 25 untreated patients were analyzed. Peak cough flow and maximum expiratory pressure were significantly higher in the steroid-treated patients. Each of the pulmonary function test variables was significantly associated with peak cough flow. The linear model that had the highest adjusted r(2) value included only 2 variables: maximum voluntary ventilation and steroid treatment, demonstrating that steroid-treated patients had peak cough flow values that were 27 L/min higher than the untreated patients. The interaction between maximum voluntary ventilation and steroid was not statistically significant, suggesting that the steroid-associated increase in peak cough flow was approximately constant over the observed range of maximum voluntary ventilation values. The effects of maximum voluntary ventilation and treatment group on peak cough flow were not confounded with the patient age.CONCLUSIONS. Long-term steroid therapy is associated with improved peak cough flow and respiratory muscle strength in patients with Duchenne muscular dystrophy. Maximum voluntary ventilation may be a useful predictor of lung function in Duchenne muscular dystrophy.