Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening

被引：285

作者：

Stick, Stephen M. ^{[1
,2
,3
]}

Brennan, Siobhain ^{[3
]}

Murray, Conor

Douglas, Tonia ^{[3
]}

von Ungern-Sternberg, Britta S.

Garratt, Luke W. ^{[3
]}

Gangell, Catherine L. ^{[3
]}

De Klerk, Nicholas ^{[3
]}

Linnane, Barry ^{[4
]}

Ranganathan, Sarath ^{[4
]}

Robinson, Phillip ^{[4
]}

Robertson, Colin ^{[4
]}

Sly, Peter D. ^{[3
]}

机构：

[1] Princess Margaret Hosp Children, Dept Resp Med, Perth, WA 6010, Australia

[2] Univ Western Australia, Sch Paediat & Child Hlth, Perth, WA 6009, Australia

[3] TVW Inst Child Hlth Res, Perth, WA, Australia

[4] Royal Childrens Hosp, Melbourne, Vic, Australia

来源：

JOURNAL OF PEDIATRICS | 2009年 / 155卷 / 05期

基金：

英国医学研究理事会;

关键词：

RESOLUTION COMPUTED-TOMOGRAPHY; PULMONARY-FUNCTION TESTS; LUNG-DISEASE; INFLAMMATION; CHEST; CT; ABNORMALITIES; ADULTS;

D O I：

10.1016/j.jpeds.2009.05.005

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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).

引用

页码：623 / U52

页数：7

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