Shmueli Einat, Gendler Yulia, Stafler Patrick, Levine Hagit, Steuer Guy, Bar-On Ophir, Blau Hannah, Prais Dario, Mei-Zahav Meir
Graub Cystic Fibrosis Center, Pulmonary Institute, Schneider Children's Medical Center of Israel, 14 Kaplan Street, Petah Tiqwa 49202, Israel.
Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel.
J Clin Med. 2023 Sep 8;12(18):5834. doi: 10.3390/jcm12185834.
Lung function deterioration in cystic fibrosis (CF) is typically measured by a decline in the forced expiratory volume in one second (FEV%), which is thought to be a late marker of lung disease. Dynamic hyperinflation (DH) is seen in obstructive lung diseases while exercising. Our aim was to assess whether DH could predict pulmonary deterioration in CF; a secondary measure was the peak VO.
A retrospective study was conducted of people with CF who performed cardiopulmonary exercise tests (CPETs) during 2012-2018. The tests were classified as those demonstrating DH non-DH. Demographic, genetic, and clinical data until 12.2022 were extracted from patient charts.
A total of 33 patients aged 10-61 years performed 41 valid CPETs with valid DH measurements; sixteen (39%) demonstrated DH. At the time of the CPETs, there was no difference in the FEV% measurements between the DH and non-DH groups (median 83.5% vs. 87.6%, respectively; = 0.174). The FEV% trend over 4 years showed a decline in the DH group compared to the non-DH group ( = 0.009). A correlation was found between DH and the lung clearance index (LCI), as well as the FEV% (r = 0.36 and = 0.019 and r = -0.55 and = 0.004, respectively). Intravenous (IV) antibiotic courses during the 4 years after the CPETs were significantly more frequent in the DH group ( = 0.046). The peak VO also correlated with the FEV% and LCI (r = 0.36 and = 0.02 and r = -0.46 and = 0.014, respectively) as well as with the IV antibiotic courses (r = -0.46 and = 0.014).
In our cohort, the DH and peak VO were both associated with lung function deterioration and more frequent pulmonary exacerbations. DH may serve as a marker to predict pulmonary deterioration in people with CF.
囊性纤维化（CF）患者的肺功能恶化通常通过一秒用力呼气量（FEV%）的下降来衡量，FEV%被认为是肺部疾病的晚期标志物。动态肺过度充气（DH）在阻塞性肺疾病患者运动时可见。我们的目的是评估DH是否能预测CF患者的肺部恶化；次要指标是峰值VO。
对2012年至2018年期间进行心肺运动试验（CPET）的CF患者进行回顾性研究。这些测试被分类为显示DH和非DH的测试。从患者病历中提取截至2022年12月的人口统计学、遗传学和临床数据。
共有33名年龄在10至61岁之间的患者进行了41次有效的CPET，并进行了有效的DH测量；16名（39%）显示有DH。在进行CPET时，DH组和非DH组的FEV%测量值没有差异（中位数分别为83.5%和87.6%；P = 0.174）。与非DH组相比，DH组4年期间的FEV%趋势呈下降趋势（P = 0.009）。发现DH与肺清除指数（LCI）以及FEV%之间存在相关性（r分别为0.36，P = 0.019；r为 -0.55，P = 0.004）。在CPET后4年期间，DH组的静脉用（IV）抗生素疗程明显更频繁（P = 0.046）。峰值VO也与FEV%和LCI相关（r分别为0.36，P = 0.02；r为 -0.46，P = 0.014），以及与IV抗生素疗程相关（r为 -0.46，P = 0.014）。
在我们的队列中，DH和峰值VO均与肺功能恶化和更频繁的肺部加重相关。DH可能作为预测CF患者肺部恶化的标志物。
