Peetsold Marieke G, Vonk-Noordegraaf Anton, Heij Hugo H, Gemke Reinoud J B J
Department of Pediatrics, VU University Medical Center, Amsterdam, The Netherlands.
Pediatr Pulmonol. 2007 Apr;42(4):325-31. doi: 10.1002/ppul.20579.
Congenital diaphragmatic hernia (CDH) is accompanied by pulmonary hypoplasia and structural abnormalities of the pulmonary vascular bed. It is unknown whether pulmonary function, exercise capacity, and gas exchange during exercise are impaired in adult CDH survivors. The objective of this study was to assess the long-term pulmonary function, exercise capacity, and gas exchange during exercise and relate these findings with quality of life. Of the 23 patients eligible for this study, 12 adult CDH survivors (mean age, 24.3 +/- 4.1 years) with high-risk CDH agreed to participate. Pulmonary function tests, diffusion capacity, and a cardiopulmonary exercise test (CPET) were performed. The FEV1 (mean z-score +/- SD; -1.30 +/- 1.37), FEF25-75% (-1.49 +/- 1.14), and the KCO (-1.03 +/- 1.24) were found to be lower in CDH survivors. The RV/TLC ratio (28.2% +/- 5.0%) was found to be higher. Despite these abnormalities, percent predicted work load (102% +/- 17.2%) and percent predicted maximal oxygen uptake (90.8% +/- 18.9%) were normal in most of the patients. The quality of life of CDH survivors, assessed with the SF-36 questionnaire, is comparable to the general population. Comparison of participants to non-participants did not reveal significant differences in clinical characteristics. In this first study assessing pulmonary function in adult survivors of CDH, mild airway obstruction was observed in most of the patients together with a slightly reduced diffusion capacity for CO. Exercise capacity and gas exchange parameters were normal in this group, indicating that patients do not have a physical impairment, as reflected by a normal quality of life of CDH patients.
先天性膈疝（CDH）伴有肺发育不全和肺血管床结构异常。目前尚不清楚成年CDH幸存者的肺功能、运动能力以及运动期间的气体交换是否受损。本研究的目的是评估长期肺功能、运动能力以及运动期间的气体交换，并将这些结果与生活质量相关联。在符合本研究条件的23例患者中，12例高危CDH成年幸存者（平均年龄24.3±4.1岁）同意参与。进行了肺功能测试、弥散功能测试和心肺运动试验（CPET）。发现CDH幸存者的第一秒用力呼气容积（FEV1，平均z评分±标准差；-1.30±1.37）、25%~75%用力呼气流量（FEF25-75%，-1.49±1.14）和一氧化碳弥散系数（KCO，-1.03±1.24）较低。残气量/肺总量比值（RV/TLC，28.2%±5.0%）较高。尽管存在这些异常，但大多数患者的预计工作负荷百分比（102%±17.2%）和预计最大摄氧量百分比（90.8%±18.9%）正常。用SF-36问卷评估的CDH幸存者的生活质量与一般人群相当。参与者与非参与者的临床特征比较未发现显著差异。在这项评估成年CDH幸存者肺功能的首次研究中，大多数患者存在轻度气道阻塞，同时一氧化碳弥散能力略有降低。该组患者的运动能力和气体交换参数正常，表明患者没有身体损伤，这一点在CDH患者正常的生活质量中得到了体现。
