Airway remodelling in children with cystic fibrosis
Airway remodelling in children with cystic fibrosis
复制标题
DOI：
10.1136/thx.2006.074641
复制
发表时间：
2007-12-01
期刊：
影响因子：
10
通讯作者：
Davies, Jane C.
中科院分区：
文献类型：
--
作者：
Hilliard, Tom N.;Regamey, Nicolas;Davies, Jane C.
背景：在早期囊性纤维化（CF）肺疾病中，气道结构改变和炎症之间的关系尚不清楚。进行了一项研究，以确定与适当的疾病和健康对照相比，CF儿童气道重塑的变化。对43例CF患儿进行了支气管肺泡灌洗和支气管内活检（年龄0.3 - 16.8岁），7名儿童患有原发性纤毛运动障碍（PCD），26例有慢性呼吸道症状（CRS）的儿童接受了反复感染和/或咳嗽的调查，7例对照儿童没有下呼吸道症状。检测支气管肺泡灌洗液（BALF）中炎性细胞、细胞因子、蛋白酶和基质成分。网状基底膜（RBM）的厚度进行了测量活检标本，使用光学microscopic.Results：弹性蛋白，糖胺聚糖和胶原蛋白的浓度增加，发现在BALF中的儿童CF与CRS组和对照组相比，每个年龄，中性粒细胞计数和蛋白酶（弹性蛋白酶活性和基质金属蛋白酶-9（MMP-9）浓度）呈正相关。CF组中，其中某些指标与肺功能（1秒用力呼气量）呈显着负相关（弹性蛋白：r =-0.45，p
Background: The relationship between airway structural changes and inflammation is unclear in early cystic fibrosis (CF) lung disease. A study was undertaken to determine changes in airway remodelling in children with CF compared with appropriate disease and healthy controls.Methods: Bronchoalveolar lavage and endobronchial biopsy were performed in a cross-sectional study of 43 children with CF (aged 0.3 - 16.8 years), 7 children with primary ciliary dyskinesia (PCD), 26 with chronic respiratory symptoms (CRS) investigated for recurrent infection and/or cough and 7 control children with no lower airway symptoms. Inflammatory cells, cytokines, proteases and matrix constituents were measured in bronchoalveolar lavage fluid (BALF). Reticular basement membrane (RBM) thickness was measured on biopsy specimens using light microscopy.Results: Increased concentrations of elastin, glycosaminoglycans and collagen were found in BALF from children with CF compared with the CRS group and controls, each correlating positively with age, neutrophil count and proteases (elastase activity and matrix metalloproteinase-9 (MMP-9) concentration). There were significant negative correlations between certain of these and pulmonary function (forced expiratory volume in 1 s) in the CF group (elastin: r = -0.45, p