TY - T1的气道突发的定量分析truction in lymphangioleiomyomatosis JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01965-2019 VL - 56 IS - 1 SP - 1901965 AU - Verleden, Stijn E. AU - Vanstapel, Arno AU - De Sadeleer, Laurens AU - Weynand, Birgit AU - Boone, Matthieu AU - Verbeken, Erik AU - Piloni, Davide AU - Van Raemdonck, Dirk AU - Ackermann, Maximilian AU - Jonigk, Danny D. AU - Verschakelen, Johny AU - Wuyts, Wim A. Y1 - 2020/07/01 UR - //www.qdcxjkg.com/content/56/1/1901965.abstract N2 - Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease with progressive pulmonary function loss caused by progressively proliferating LAM cells. The degree of airway obstruction has not been well investigated within the pathogenesis of LAM.Using a combination of ex vivo computed tomography (CT), microCT and histology, the site and nature of airway obstruction in LAM explant lungs was compared with matched control lungs (n=5 each). The total number of airways per generation, total airway counts, terminal bronchioles number and surface density were compared in LAM versus control.Ex vivo CT analysis demonstrated a reduced number of airways from generation 7 on (p<0.0001) in LAM compared with control, whereas whole-lung microCT analysis confirmed the three- to four-fold reduction in the number of airways. Specimen microCT analysis further demonstrated a four-fold decrease in the number of terminal bronchioles (p=0.0079) and a decreased surface density (p=0.0079). Serial microCT and histology images directly showed the loss of functional airways by collapse of airways on the cysts and filling of the airway by exudate.LAM lungs show a three- to four-fold decrease in the number of (small) airways, caused by cystic destruction which is the likely culprit for the progressive loss of pulmonary function.This study demonstrates a 4-fold reduction in the number of airways and terminal bronchioles in end-stage LAM lungs using a combination of CT, microCT and histopathology, compared to a matched control group http://bit.ly/2tBTiJy ER -