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Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis
Journal of Cystic Fibrosis ( IF 5.2 ) Pub Date : 2020-12-01 , DOI: 10.1016/j.jcf.2020.11.019
Matthew M Willmering 1 , David J Roach 1 , Elizabeth L Kramer 2 , Laura L Walkup 3 , Zackary I Cleveland 3 , Jason C Woods 4
Affiliation  

BACKGROUND Two functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes. METHODS Spirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record. RESULTS MBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry (<30%). UTE MRI suggests increased odds of bronchial changes when mucus plugging is present in the same lobe. MBW and 129Xe MRI correlated best with mucus plugging, while spirometry correlated best with consolidations. Bronchial abnormalities were associated with future pulmonary exacerbations. CONCLUSIONS MBW, 129Xe MRI, and UTE MRI are more sensitive for detection of pediatric CF lung disease when compared to spirometry. MBW and 129Xe MRI correlated with structural abnormalities which occur in early CF disease, suggesting MBW and 129Xe MRI are valuable tools in mild CF lung disease that can guide clinical decision making.

中文翻译:

小儿囊性纤维化的敏感结构和功能测量以及 1 年肺部结果

背景 与传统肺活量测定相比,两种功能测量(多次呼吸冲洗 [MBW] 和超极化 129Xe 通气磁共振成像 [129Xe MRI])已被证明对囊性纤维化 (CF) 肺阻塞更敏感。然而,功能技术可能对不同的潜在结构异常敏感。本研究的目的是确定这些功能标志物、它们的病理生理学和 1 年临床结果之间的关系。方法 在对 27 名未开始 CFTR 调节剂治疗的儿童 CF 患者(年龄 11.5±5.0)进行同一天评估时,获得了肺活量测定、MBW、129Xe MRI 和超短回波时间 (UTE) MRI。UTE MRI 对通过肺活量计、MBW 和 129Xe MRI 获得的结构异常和功能指标进行评分。从电子病历中获得 1 年结果(ΔFEV1 和肺部恶化),在此期间约 50% 开始调节剂治疗。结果与肺活量测定法(<30%)相比,MBW、129Xe MRI 和 UTE MRI 在更多受试者(>78%)中检测到有临床意义的疾病。UTE MRI 表明,当粘液堵塞出现在同一肺叶时,支气管改变的几率会增加。MBW 和 129Xe MRI 与粘液堵塞的相关性最好,而肺活量测定与巩固的相关性最好。支气管异常与未来的肺部恶化有关。结论 MBW、129Xe MRI 和 UTE MRI 在检测小儿 CF 肺病方面比肺活量测定法更敏感。MBW 和 129Xe MRI 与早期 CF 疾病中发生的结构异常相关,
更新日期:2020-12-01
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