Stick Stephen M, Brennan Siobhain, Murray Conor, Douglas Tonia, von Ungern-Sternberg Britta S, Garratt Luke W, Gangell Catherine L, De Klerk Nicholas, Linnane Barry, Ranganathan Sarath, Robinson Phillip, Robertson Colin, Sly Peter D
Department of Respiratory Medicine, Princess Margaret Hospital for Children, School of Paediatrics and Child Health, University of Western Australia, Melbourne, Australia.
J Pediatr. 2009 Nov;155(5):623-8.e1. doi: 10.1016/j.jpeds.2009.05.005. Epub 2009 Jul 19.
To determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.
Children were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.
The prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).
Pulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.
确定新生儿筛查（NBS）后诊断为囊性纤维化（CF）的幼儿支气管扩张的患病率，以及支气管扩张与肺部炎症和感染的关系。
儿童在NBS后被诊断为CF。在麻醉状态下进行计算机断层扫描和支气管肺泡灌洗（n = 96）。分析扫描结果以确定异常的存在和程度。
支气管扩张的患病率为22%，且随年龄增长而增加（P = .001）。与支气管扩张相关的因素包括绝对中性粒细胞计数（P = .03）、中性粒细胞弹性蛋白酶浓度（P = .001）和铜绿假单胞菌感染（P = .03）。
CF婴幼儿肺部异常常见，与嗜中性粒细胞炎症及铜绿假单胞菌感染有关。目前CF婴幼儿护理模式未能预防呼吸后遗症。支气管扩张是一个具有临床相关性的终点指标，可用于在NBS后CF诊断后不久开始的干预试验。
