Young A C, Wilson J W, Kotsimbos T C, Naughton M T
Department of Allergy Immunology and Respiratory Medicine, The Alfred Hospital, Commercial Rd, Prahran, Melbourne, VIC, 3181, Australia.
Thorax. 2008 Jan;63(1):72-7. doi: 10.1136/thx.2007.082602. Epub 2007 Aug 3.
The clinical benefits of domiciliary non-invasive positive pressure ventilation (NIV) have not been established in cystic fibrosis (CF). We studied the effects of nocturnal NIV on quality of life (QoL), functional and physiological outcomes in CF subjects with awake hypercapnia (arterial carbon dioxide pressure PaCO2>45 mm Hg).
In a randomised, placebo controlled, crossover study, eight subjects with CF, mean (SD) age 37 (8) years, body mass index 21.1 (2.6) kg/m2, forced expiratory volume in 1 s 35 (8)% predicted and PaCO2 52 (4) mm Hg received 6 weeks of nocturnal (1) air (placebo), (2) oxygen and (3) NIV. The primary outcome measures were CF specific QoL, daytime sleepiness and exertional dyspnoea. Secondary outcome measures were awake and asleep gas exchange, sleep architecture, lung function and peak exercise capacity.
Compared with air, NIV improved the chest symptom score in the CF QoL Questionnaire (mean difference 10; 95% CI 5 to 16; p = 0.002) and the transitional dyspnoea index score (mean difference 3.1; 95% CI 1.2-5.0; p = 0.01). It reduced maximum nocturnal pressure of transcutaneous CO2 (PtcCO2 mean difference -17 mm Hg; 95% CI -7 to -28 mm Hg; p = 0.005) and increased exercise performance on the Modified Shuttle Test (mean difference 83 m; 95% CI 21 to 144 m; p = 0.02). NIV did not improve sleep architecture, lung function or awake PaCO2.
6 weeks of nocturnal NIV improves chest symptoms, exertional dyspnoea, nocturnal hypoventilation and peak exercise capacity in adult patients with stable CF with awake hypercapnia. Further studies are required to determine whether or not NIV can improve survival.
家庭无创正压通气（NIV）在囊性纤维化（CF）中的临床益处尚未得到证实。我们研究了夜间NIV对清醒时高碳酸血症（动脉二氧化碳分压PaCO2>45 mmHg）的CF患者生活质量（QoL）、功能和生理结局的影响。
在一项随机、安慰剂对照、交叉研究中，8名CF患者，平均（标准差）年龄37（8）岁，体重指数21.1（2.6）kg/m2，第1秒用力呼气量为预测值的35（8）%，PaCO2为52（4）mmHg，接受了为期6周的夜间（1）空气（安慰剂）、（2）氧气和（3）NIV治疗。主要结局指标为CF特异性QoL、日间嗜睡和运动性呼吸困难。次要结局指标为清醒和睡眠时的气体交换、睡眠结构、肺功能和峰值运动能力。
与空气相比，NIV改善了CF生活质量问卷中的胸部症状评分（平均差异10；95%CI 5至16；p = 0.002）和过渡性呼吸困难指数评分（平均差异3.1；95%CI 1.2 - 5.0；p = 0.01）。它降低了夜间经皮二氧化碳最大压力（PtcCO2平均差异 - 17 mmHg；95%CI - 7至 - 28 mmHg；p = 0.005），并提高了改良往返试验中的运动表现（平均差异83 m；95%CI 21至144 m；p = 0.02）。NIV未改善睡眠结构、肺功能或清醒时的PaCO2。
6周的夜间NIV可改善清醒时高碳酸血症的稳定CF成年患者的胸部症状、运动性呼吸困难、夜间通气不足和峰值运动能力。需要进一步研究以确定NIV是否能提高生存率。
