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Leveraging early markers of cystic fibrosis structural lung disease to improve outcomes
European Respiratory Journal ( IF 24.3 ) Pub Date : 2020-04-01 , DOI: 10.1183/13993003.00105-2020
Patrick A. Flume , Donald R. VanDevanter

The natural history of cystic fibrosis (CF) lung disease has been described as a complex process of obstruction, infection, and inflammation, all contributing to airways injury and eventual structural disease (i.e. bronchiectasis). Consequences of the CF genetic abnormality begin in utero and are the basis for newborn screening for elevated levels of circulating immunoreactive trypsinogen (IRT) [1]. Thus, it should be no surprise that the process of airway obstruction begins immediately at birth. Ample cross-sectional evidence has been published of airway obstruction, inflammation, bacterial infection, bronchial wall thickening, air trapping and bronchiectasis in the first year of life for infants with CF [2]. Wijker and colleagues have demonstrated the link of inflammation and obstruction to eventual structural disease in CF. Now we may have another clinical endpoint by which we can judge the impact of early interventions. http://bit.ly/2v5A5Qw

中文翻译:

利用囊性纤维化结构性肺病的早期标志物来改善预后

囊性纤维化 (CF) 肺病的自然病程已被描述为阻塞、感染和炎症的复杂过程,所有这些过程都会导致气道损伤和最终的结构性疾病(即支气管扩张)。CF 基因异常的后果始于子宫内,是新生儿筛查循环免疫反应性胰蛋白酶原 (IRT) 水平升高的基础 [1]。因此,气道阻塞的过程在出生时立即开始也就不足为奇了。已经发表了大量关于 CF 婴儿在出生后第一年气道阻塞、炎症、细菌感染、支气管壁增厚、空气滞留和支气管扩张的横断面证据[2]。Wijker 及其同事已经证明炎症和阻塞与 CF 中最终的结构性疾病之间存在联系。现在我们可能有另一个临床终点,我们可以通过它来判断早期干预的影响。http://bit.ly/2v5A5Qw
更新日期:2020-04-01
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