Respiratory Medicine, Children's Health Queensland Hospital and Health Service, South Birsbane, Queensland, Australia.
Respiratory Medicine, Children's Health Queensland Hospital and Health Service, South Birsbane, Queensland, Australia
BMJ Open Respir Res. 2020 Feb;7(1). doi: 10.1136/bmjresp-2019-000517.
Use of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival.
To describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between and subjects.
A retrospective cohort of adolescents with DMD initiating NIV over 10 years was conducted. Serial LF before and after NIV initiation was collated. Use of systemic glucocorticosteroids, adherence to NIV and presence of cardiac disease were assessed.
Twenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47-17.96). Nine were and eight were . Indications for NIV were apnoea-hypopnoea index >5 and/or nocturnal hypoventilation. LF is better (forced vital capacity (FVC) z-score -3.26 vs -5.41, p < 0.02) and decline slower (FVC z-score -0.58 per annum (pa) vs -0.68 pa, p<0.001) in compared with subjects. Following NIV initiation, FVC z-score decline slowed for the whole (-0.72 pa (95% CI -0.79 to 0.64) to -0.46 pa (95% CI -0.54 to 0.38) p < 0.001) and groups (-0.74 (95% CI -0.85 to 0.63) to -0.44 pa (95% CI -0.56 to 0.32) p < 0.001) but accelerated in the group (-0.56 (95% CI -0.70 to 0.42) to -0.75 pa (95% CI -0.89 to 0.61) p < 0.001). Adherence to NIV and cardiac disease did not impact decline.
Overall, LF decline is reduced on NIV. patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in which requires further prospective research.
随着杜氏肌营养不良症（DMD）患者生存时间的延长，无创通气（NIV）的应用也在增加。
描述开始使用 NIV 治疗的 DMD 青少年患者的肺功能（LF）变化，并评估不同年龄段患者之间的差异。
对 10 年内开始使用 NIV 的 DMD 青少年患者进行回顾性队列研究。整理分析患者开始使用 NIV 前后的系列 LF 数据。评估系统性糖皮质激素的使用、NIV 的依从性以及是否存在心脏疾病。
29 名男性开始使用 NIV，中位年龄 14.66 岁（IQR 2.35，10.47-17.96）。其中 9 人为 ，8 人为 。NIV 的适应证为呼吸暂停低通气指数（apnoea-hypopnoea index，AHI）>5 和/或夜间低通气。与 组相比，组患者的 LF 更好（用力肺活量（forced vital capacity，FVC）z 评分-3.26 比-5.41，p<0.02），下降速度更慢（FVC z 评分-0.58 每年（per annum，pa）比-0.68 pa，p<0.001）。开始使用 NIV 后，整个（-0.72 pa（95%CI -0.79 至 0.64）至-0.46 pa（95%CI -0.54 至 0.38），p<0.001）和 组（-0.74（95%CI -0.85 至 0.63）至-0.44 pa（95%CI -0.56 至 0.32），p<0.001）的 FVC z 评分下降速度均减慢，但 组的 FVC z 评分下降速度加快（-0.56 pa（95%CI -0.70 至 0.42）至-0.75 pa（95%CI -0.89 至 0.61），p<0.001）。NIV 的依从性和心脏疾病对 LF 下降没有影响。
总体而言，使用 NIV 后 LF 下降速度减慢。 患者的 LF 更低，下降速度更快，但在开始使用 NIV 后下降速度减慢。 患者在使用 NIV 时，LF 下降速度加快，需要进一步进行前瞻性研究。
