Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy
Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy
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DOI：
10.5535/arm.2016.40.1.74
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发表时间：
2016-02-01
影响因子：
1.3
通讯作者：
Lee, Kil Chan
中科院分区：
文献类型：
--
作者：
Cho, Han Eol;Lee, Jang Woo;Lee, Kil Chan
目的 评估肌萎缩侧索硬化症（ALS）、杜兴氏肌营养不良症（DMD）和强直性肌营养不良症（MMD）患者在通气功能不全发作时的肺功能。方法本回顾性研究纳入无创正压通气前在江南塞弗兰斯医院康复医学科定期门诊随访的ALS、DMD和MMD患者。 （NIPPV）。患者入组时间为2001年8月至2014年3月。如果患者出现通气功能不全，则接受NIPPV治疗，随后测量肺功能。结果本研究共纳入94例DMD患者、41例ALS患者和21例MMD患者。 MMD 组的平均 SpO(2) 低于其他两组。 DMD 和 ALS 患者仰卧位的平均用力肺活量 (FVC) 大约低至 20% 左右，而 MMD 患者则高出 10%。 ALS 患者仰卧位的用力肺活量显着低于坐位。 MMD 组的最大吹气量、无辅助咳嗽峰值流量、最大吸气压 (MIP) 和最大呼气压 (MEP) 显着高于其他组。 DMD患者的MEP明显最低，其次是ALS、MMD患者。 结论 疾病特异性肺功能值，包括FVC、MEP和MIP，可以准确评估ALS、DMD和MMD患者通气功能不全的发生情况。
Objective To evaluate pulmonary functions of patients with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and myotonic muscular dystrophy (MMD) at the onset of ventilatory insufficiency.Methods This retrospective study included ALS, DMD, and MMD patients with regular outpatient clinic follow-up in the Department of Rehabilitation Medicine at Gangnam Severance Hospital before the application of non-invasive positive pressure ventilation (NIPPV). The patients were enrolled from August 2001 to March 2014. If patients experienced ventilatory insufficiency, they were treated with NIPPV, and their pulmonary functions were subsequently measured.Results Ninety-four DMD patients, 41 ALS patients, and 21 MMD patients were included in the study. The mean SpO(2) was lower in the MMD group than in the other two groups. The mean forced vital capacity (FVC) in the supine position was approximately low to mid 20% on average in DMD and ALS patients, whereas it was 10% higher in MMD patients. ALS patients showed a significantly lower FVC in the supine position than in the sitting position. Maximal insufflation capacity, unassisted peak cough flow, maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP) were significantly higher in MMD group than in the other groups. MEP was significantly the lowest in DMD patients, followed by in ALS, and MMD patients, in order.Conclusion Disease-specific values of pulmonary function, including FVC, MEP, and MIP, can be accurately used to assess the onset of ventilatory insufficiency in patients with ALS, DMD, and MMD.