Early lung disease in young children with primary ciliary dyskinesia

被引：54

作者：

Brown, David E. ^{[1
]}

Pittman, Jessica E. ^{[1
]}

Leigh, Margaret W. ^{[1
]}

Fordham, Lynn ^{[2
]}

Davis, Stephanie D. ^{[1
]}

机构：

[1] Univ N Carolina, Div Pulmonol, Dept Pediat, Chapel Hill, NC 27599 USA

[2] Univ N Carolina, Dept Radiol, Chapel Hill, NC 27599 USA

来源：

PEDIATRIC PULMONOLOGY | 2008年 / 43卷 / 05期

关键词：

bronchiectasis; infant; respiratory function testing; Kartagener syndrome; situs inversus;

D O I：

10.1002/ppul.20792

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Primary ciliary dyskinesia (PCD) is an autosomal recessive disease in which ciliary dysfunction leads to chronic lung, sinus, and middle ear disease. PCD is often not diagnosed until late childhood due to its presumed rarity and the technical expertise necessary for diagnosis; as such, little is known about lung disease in young children with PCD. We report on 3 young children with PCD who had evidence of lung disease on infant pulmonary function testing, bronchoscopy, and/or computed tomography (CT) of the chest before 3 years of age.

引用

页码：514 / 516

页数：3

共 7 条

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