Katz Sherri L, Barrowman Nicholas, Monsour Andrea, Su Santana, Hoey Lynda, McKim Douglas
1 Division of Respiratory Medicine, Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
2 Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.
Ann Am Thorac Soc. 2016 Feb;13(2):217-22. doi: 10.1513/AnnalsATS.201507-475BC.
Lung volume recruitment therapy slows rate of decline of lung function in neuromuscular disease, possibly due to enhanced airway clearance, reduced atelectasis, or prevention of chest wall contractures.
To determine if lung volume recruitment maintains maximal insufflation capacity (MIC), despite decline in VC.
This was a retrospective cohort study (1991-2008) of individuals with Duchenne muscular dystrophy at pediatric and adult tertiary centers. Lung volume recruitment was prescribed twice daily, according to protocol. Changes over time in MIC, VC percentage predicted, the difference between MIC and VC, maximum inspiratory and expiratory pressures, and assisted and unassisted peak cough flow (PCF) were assessed using linear mixed effects models.
Sixteen individuals, 8.6 to 33.0 years old at initiation of lung volume recruitment, with median VC percentage predicted of 13.5 (interquartile range, 8.0-20.3), were followed over a median of 6.1 years (range, 1.7-16.1 yr). MIC-VC differences were stable (change, 0.02 L/yr; P = 0.06). Post-lung volume recruitment, compared with pretreatment, rate of decline in VC decreased from 4.5% predicted/yr to 0.5% predicted/yr (P < 0.001). Maximal inspiratory and expiratory pressures were unchanged (P = 0.08, 0.59 respectively). Assisted-spontaneous PCF difference was maintained (slope, -1.59 L/min/yr, P = 0.35).
With lung volume recruitment therapy, MIC-VC differences were stable over time, indicating that respiratory system compliance remains stable, despite a loss in VC, in individuals with Duchenne muscular dystrophy. Decline in VC was significantly attenuated, and assisted PCF was maintained in a clinically effective range.
肺容积复张治疗可减缓神经肌肉疾病患者肺功能的下降速度，这可能是由于气道清除能力增强、肺不张减少或胸壁挛缩得到预防。
确定肺容积复张能否在肺活量（VC）下降的情况下维持最大吸气容量（MIC）。
这是一项对儿科和成人三级中心的杜氏肌营养不良患者进行的回顾性队列研究（1991 - 2008年）。根据方案，肺容积复张治疗每天进行两次。使用线性混合效应模型评估MIC、预测的VC百分比、MIC与VC之间的差异、最大吸气和呼气压力以及辅助和非辅助峰值咳嗽流量（PCF）随时间的变化。
16名患者在开始肺容积复张治疗时年龄为8.6至33.0岁，预测的VC百分比中位数为13.5（四分位间距，8.0 - 20.3），中位随访时间为6.1年（范围，1.7 - 16.1年）。MIC - VC差异稳定（变化，0.02升/年；P = 0.06）。与治疗前相比，肺容积复张治疗后，VC的下降速度从预测的4.5%/年降至0.5%/年（P < 0.001）。最大吸气和呼气压力无变化（P分别为0.08、0.59）。辅助 - 自主PCF差异得以维持（斜率，-1.59升/分钟/年，P = 0.35）。
采用肺容积复张治疗时，MIC - VC差异随时间保持稳定，这表明在杜氏肌营养不良患者中，尽管VC有所下降，但呼吸系统顺应性仍保持稳定。VC的下降显著减缓，且辅助PCF维持在临床有效范围内。
