Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis What Have We Learned and What Should We Do about It?

被引：97

作者：

Ranganathan, Sarath C. ^{[1
,2
,3
]}

Hall, Graham L. ^{[4
,5
,6
]}

Sly, Peter D. ^{[7
,8
]}

Stick, Stephen M. ^{[4
,5
,9
]}

Douglas, Tonia A. ^{[4
,7
]}

机构：

[1] Royal Childrens Hosp, Dept Resp Med, Melbourne, Vic, Australia

[2] Murdoch Childrens Res Inst, Infect & Immun, Melbourne, Vic, Australia

[3] Univ Melbourne, Dept Paediat, Melbourne, Vic, Australia

[4] Telethon Kids Inst, Perth, WA, Australia

[5] Univ Western Australia, Ctr Child Hlth Res, Perth, WA, Australia

[6] Curtin Univ, Sch Physiotherapy & Exercise Sci, Perth, WA, Australia

[7] Childrens Hlth Queensland, Dept Resp & Sleep Med, South Brisbane, Qld, Australia

[8] Univ Queensland, Child Hlth Res Ctr, Brisbane, Qld, Australia

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

来源：

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE | 2017年 / 195卷 / 12期

关键词：

cystic fibrosis; early lung disease; newborn screening; early intervention; PSEUDOMONAS-AERUGINOSA ACQUISITION; YOUNG-CHILDREN; COMPUTED-TOMOGRAPHY; PULMONARY INFLAMMATION; AIRWAY INFLAMMATION; CLEARANCE INDEX; DORNASE-ALPHA; VENTILATION INHOMOGENEITY; BRONCHOALVEOLAR LAVAGE; RESPIRATORY-INFECTION;

D O I：

10.1164/rccm.201606-1107CI

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and structural lung damage manifest very early in life and are prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer-term clinical trajectory for individuals with CF. This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for future clinical practice and research.

引用

页码：1567 / 1575

页数：9

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