Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States; Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States.
Division of Pulmonary Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Ave, Chicago, Illinois, 60611, United States.
J Cyst Fibros. 2023 Sep;22(5):926-932. doi: 10.1016/j.jcf.2023.01.017. Epub 2023 Feb 4.
Airway clearance therapy (ACT) with a high-frequency chest wall oscillation (HFCWO) vest is a common but time-consuming treatment. Its benefit to quality of life for cystic fibrosis (CF) patients is well established but has been questioned recently as new highly-effective modulator therapies begin to change the treatment landscape. Xe ventilation MRI has been shown to be very sensitive to lung obstruction in mild CF disease, making it an ideal tool to identify and quantify subtle, regional changes.
20 CF patients (ages 20.7 ± 5.1 years) refrained from performing ACT before arriving for a single-day visit. Multiple-breath washout (MBW), spirometry, Xe MRI, and ultrashort echo-time (UTE) MRI were obtained twice-before and after patients performed ACT using their prescribed HFCWO vests (average 4.7 ± 0.5 h). UTE MRIs were scored for structural abnormalities, and standard functional metrics were obtained from MBW, spirometry, and Xe MRI-FEV, LCI, and VDP, respectively.
Spirometry and Xe MRI detected significant improvements in lung function post-ACT. 15/20 patients showed improvements from a baseline median of 92% FEV. Similarly, 16/20 patients showed improvements in Xe MRI from a baseline median of 15.2% VDP. Average individual changes were +2.6% in FEV and -1.3% in VDP, but without spatial correlations to easily-identifiable causative structural defects (e.g. mucus plugs or bronchiectasis) on UTE MRI.
Lung function improved after a single instance of HFCWO-vest ACT and was detectable by spirometry and Xe MRI. The only common structural abnormalities were mucus plugs, which corresponded to ventilation defects, but ventilation defects were often present without visible abnormalities.
高频胸壁振荡（HFCWO）背心式气道廓清治疗是一种常见但耗时的治疗方法。其对囊性纤维化（CF）患者生活质量的益处已得到充分证实，但随着新型高效调节剂治疗方法的出现，改变了治疗格局，最近这一益处受到了质疑。Xe 通气 MRI 已被证明对轻度 CF 疾病中的肺阻塞非常敏感，使其成为识别和量化细微、区域性变化的理想工具。
20 名 CF 患者（年龄 20.7±5.1 岁）在单日内就诊前停止进行 ACT。进行了多次呼吸冲洗（MBW）、肺活量测定、Xe MRI 和超短回波时间（UTE）MRI 检查，两次检查分别在患者使用规定的 HFCWO 背心（平均 4.7±0.5 h）进行 ACT 之前和之后进行。UTE MRI 对结构异常进行评分，MBW、肺活量测定和 Xe MRI 分别获得标准功能指标，包括 FEV、LCI 和 VDP。
肺活量测定和 Xe MRI 在 ACT 后检测到肺功能显著改善。15/20 名患者的 FEV 基线中位数从 92%提高。同样，16/20 名患者的 Xe MRI 基线中位数从 15.2%VDP 提高。FEV 的平均个体变化为+2.6%，VDP 变化为-1.3%，但与 UTE MRI 上容易识别的因果结构缺陷（如黏液栓或支气管扩张）没有空间相关性。
HFCWO 背心式 ACT 单次治疗后肺功能改善，可通过肺活量测定和 Xe MRI 检测到。唯一常见的结构异常是黏液栓，其对应通气缺陷，但通气缺陷常常存在而无可见异常。
