Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening
Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening
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DOI：
10.1016/j.jpeds.2009.05.005
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发表时间：
2009-11-01
影响因子：
5.1
通讯作者：
Sly, Peter D.
中科院分区：
文献类型：
--
作者：
Stick, Stephen M.;Brennan, Siobhain;Sly, Peter D.
目的探讨新生儿筛查（NBS）后确诊的囊性纤维化（CF）患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。在麻醉下进行计算机断层扫描和支气管肺泡灌洗（n = 96）。结果支气管扩张症的患病率为22%，并随年龄增长而增加（P = 0.001）。与支气管扩张相关的因素包括中性粒细胞绝对计数（P = 0.03）、中性粒细胞弹性蛋白酶浓度（P = 0.001）和铜绿假单胞菌感染（P = 0.03）。目前CF婴儿的护理模式未能预防呼吸系统后遗症。支气管扩张是一个临床相关终点，可用于NBS诊断CF后不久开始的干预试验。（J Pediatr 2009; 155：623-8）。
Objectives 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.Study design 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.Results 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).Conclusions 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. (J Pediatr 2009; 155: 623-8).