Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia; Discipline of Child and Adolescent Health, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.
Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia.
Paediatr Respir Rev. 2024 Mar;49:9-13. doi: 10.1016/j.prrv.2023.08.002. Epub 2023 Aug 11.
To explore the relationship between postural changes in lung function and polysomnography (PSG) in children with Duchenne muscular dystrophy (DMD).
In this prospective cross-sectional study, children with DMD performed spirometry in sitting and supine positions. A control group of age and gender matched healthy children also underwent postural lung function testing. PSG was performed within six months of spirometry.
Seventeen children with DMD, aged 12.3 ± 3 years performed sitting spirometry. 14 (84%) performed acceptable spirometry in the supine position. Mean FEV and FVC were 77% (SD ± 22) and 74% (SD ± 20.4) respectively, with mean% ΔFVC 9% (SD ± 11) (range 2% to 20%), and was significantly greater than healthy controls 4% (n = 30, SD ± 3, P < 0.001). PSG data on the 14 DMD children with acceptable supine spirometry showed total AHI 6.9 ± 5.9/hour (0.3 to 29), obstructive AHI 5.2 ± 4.0/hour (0.2 to 10), and REM AHI 14.1 ± -5.3/hour (0.1 to 34.7). ΔFVC(sit-sup) had poor correlation with hypoventilation on polysomnography.
Children with DMD and mild restrictive lung disease showed greater postural changes in spirometry than healthy controls but lower supine spirometry was not predictive of sleep hypoventilation.
探讨杜氏肌营养不良症(DMD)患儿肺功能体位变化与多导睡眠图(PSG)的关系。
在这项前瞻性的病例对照研究中,DMD 患儿分别在坐位和仰卧位下进行肺功能检查。同时,选择年龄和性别相匹配的健康儿童作为对照组,也进行体位肺功能检查。所有研究对象均在肺功能检查后 6 个月内进行 PSG。
17 例年龄 12.3±3 岁的 DMD 患儿进行了坐位肺功能检查,14 例(84%)在仰卧位下可进行可接受的肺功能检查。仰卧位时,FEV1 和 FVC 的平均值分别为 77%(±22)和 74%(±20.4),平均%ΔFVC 为 9%(±11)(范围 2%至 20%),显著高于健康对照组的 4%(n=30,±3,P<0.001)。在可接受仰卧位肺功能检查的 14 例 DMD 患儿中,PSG 数据显示总 AHI 为 6.9±5.9/h(0.3 至 29),阻塞性 AHI 为 5.2±4.0/h(0.2 至 10),和 REM AHI 为 14.1±-5.3/h(0.1 至 34.7)。ΔFVC(坐位-仰卧位)与 PSG 上的通气不足相关性较差。
DMD 患儿存在轻度限制性肺疾病,其肺功能的体位变化大于健康对照组,但仰卧位下肺功能较低并不能预测睡眠通气不足。