Cho Han Eol, Lee Jang Woo, Kang Seong Woong, Choi Won Ah, Oh Hyeonjun, Lee Kil Chan
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea.
Department Rehabilitation Medicine and Research Institute of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2016 Feb;40(1):74-80. doi: 10.5535/arm.2016.40.1.74. Epub 2016 Feb 26.
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.
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.
Ninety-four DMD patients, 41 ALS patients, and 21 MMD patients were included in the study. The mean SpO2 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.
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.
评估肌萎缩侧索硬化症（ALS）、杜氏肌营养不良症（DMD）和强直性肌营养不良症（MMD）患者在出现通气功能不全时的肺功能。
这项回顾性研究纳入了江南Severance医院康复医学科在应用无创正压通气（NIPPV）之前定期门诊随访的ALS、DMD和MMD患者。患者纳入时间为2001年8月至2014年3月。如果患者出现通气功能不全，就给予NIPPV治疗，随后测量其肺功能。
94例DMD患者、41例ALS患者和21例MMD患者纳入本研究。MMD组的平均SpO2低于其他两组。DMD和ALS患者仰卧位时的平均用力肺活量（FVC）平均约为20%的低至中度水平，而MMD患者则高10%。ALS患者仰卧位时的FVC显著低于坐位时。MMD组的最大吸气量、自主咳嗽峰值流速、最大吸气压力（MIP）和最大呼气压力（MEP）显著高于其他组。DMD患者的MEP显著最低，其次是ALS患者，MMD患者的MEP最高。
包括FVC、MEP和MIP在内的特定疾病肺功能值可准确用于评估ALS、DMD和MMD患者通气功能不全的发生。
