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主动肺容积募集以保留杜氏肌营养不良症患者的肺活量

Active lung volume recruitment to preserve vital capacity in Duchenne muscular dystrophy.

作者信息

Chiou Michael, Bach John R, Jethani Lavina, Gallagher Michael F

机构信息

Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, 07103 Newark, USA.

出版信息

J Rehabil Med. 2017 Jan 19;49(1):49-53. doi: 10.2340/16501977-2144.

DOI:10.2340/16501977-2144
PMID:27630091
Abstract

OBJECTIVE

To consider the effect of active lung volume recruitment ("air stacking") on rate of decline in vital capacity.

DESIGN

Retrospective cross-sectional design.

PATIENTS

People with Duchenne muscular dystrophy.

METHODS

Vital capacity was measured at every patient visit and then graphed. Air stacking using volume-preset ventilation or manual resuscitator bag was introduced to all patients after their vital capacity plateaued (reached a lifetime maximum).

RESULTS

For 151 consecutive patients with multi-year data, the 1-year rate of greatest decline in post-plateau vital capacity was 104 ml (8.8%)/year and occurred from age 20 to 21 years. For 53 patients with multi-visit data for whom air stacking was begun at the immediate post-plateau visit, the 1-year rate of greatest decline in vital capacity was 118 ml (8.5%) and occurred from age 20 to 21 years. Between annual visits, vital capacity and maximum insufflation capacity increased 26.4% and 43.3% of the time, respectively. The peak of maximal vital capacity decline occurred more than 5 years later than previously reported without air stacking.

CONCLUSION

For patients with Duchenne muscular dystrophy, active lung volume recruitment may help to preserve vital capacity. Effects on post-plateau vital capacity may be a useful outcome measure for therapeutic trials.

摘要

目的

探讨主动肺容积募集(“空气堆叠”)对肺活量下降速率的影响。

设计

回顾性横断面设计。

患者

杜氏肌营养不良症患者。

方法

在每次患者就诊时测量肺活量,然后绘制图表。在所有患者的肺活量达到平台期(达到终生最大值)后,采用容积预设通气或手动复苏袋进行空气堆叠。

结果

对于151例有多年数据的连续患者,平台期后肺活量最大下降的1年速率为每年104毫升(8.8%),发生在20至21岁之间。对于53例在平台期后立即开始进行空气堆叠且有多次就诊数据的患者,肺活量最大下降的1年速率为118毫升(8.5%),发生在20至21岁之间。在年度就诊之间,肺活量和最大吸气量分别有26.4%和43.3%的时间增加。最大肺活量下降的峰值比之前报道的无空气堆叠情况晚了5年多出现。

结论

对于杜氏肌营养不良症患者,主动肺容积募集可能有助于维持肺活量。对平台期后肺活量的影响可能是治疗试验的一个有用的结局指标。

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