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囊性纤维化患儿从婴儿期到学龄期肺清除指数的长期变化。

Long-term course of lung clearance index between infancy and school-age in cystic fibrosis subjects.

机构信息

Division of Respiratory Medicine, Department of Paediatrics, University Children's Hospital of Bern, Inselspital, 3010 Bern, Switzerland.

出版信息

J Cyst Fibros. 2011 Dec;10(6):487-90. doi: 10.1016/j.jcf.2011.07.006. Epub 2011 Aug 10.

DOI:10.1016/j.jcf.2011.07.006
PMID:21835702
Abstract

Multiple breath washout (MBW) measurements have recently been shown to be sensitive for detection of early cystic fibrosis (CF) lung disease, with the lung clearance index (LCI) being the most common measure for ventilation inhomogeneity. The aim of this observational study was to describe the longitudinal course of LCI from time of clinical diagnosis during infancy to school-age in eleven children with CF. Elevated LCI during infancy was present in seven subjects, especially in those with later clinical diagnosis. Tracking of LCI at follow-up was evident only in the four most severe cases. We provide the first longitudinal data describing the long-term course of LCI in a small group of infants with CF. Our findings support the clinical usefulness of MBW measurements to detect and monitor early lung disease in children with CF already present shortly after clinical diagnosis.

摘要

多次呼吸冲洗(MBW)测量最近已被证明对早期囊性纤维化(CF)肺病的检测具有敏感性,其中肺清除指数(LCI)是最常用的通气不均匀性测量指标。本观察性研究的目的是描述 11 名 CF 患儿从婴儿期临床诊断到学龄期的 LCI 纵向变化过程。7 名受试者在婴儿期存在 LCI 升高,尤其是临床诊断较晚的受试者。只有在 4 例最严重的病例中才出现 LCI 随访追踪的现象。我们提供了第一个描述 CF 婴儿中 LCI 长期变化过程的纵向数据。我们的研究结果支持 MBW 测量在儿童 CF 中用于检测和监测早期肺疾病的临床应用,这种检测可在临床诊断后不久进行。

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ERJ Open Res. 2018 Feb 23;4(1). doi: 10.1183/23120541.00012-2017. eCollection 2018 Jan.
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Eur Respir J. 2017 Oct 5;50(4). doi: 10.1183/13993003.00433-2017. Print 2017 Oct.
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