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尽管囊性纤维化患者肺功能稳定,但高分辨率计算机断层扫描显示仍有进行性损伤。

Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis.

作者信息

de Jong P A, Nakano Y, Lequin M H, Mayo J R, Woods R, Paré P D, Tiddens H A W M

机构信息

Dept of Paediatric Pulmonology, Sophia Children's Hospital, Erasmus Medical Centre Rotterdam, Rotterdam, The Netherlands.

出版信息

Eur Respir J. 2004 Jan;23(1):93-7. doi: 10.1183/09031936.03.00006603.

DOI:10.1183/09031936.03.00006603
PMID:14738238
Abstract

For effective clinical management of cystic fibrosis (CF) lung disease it is important to closely monitor the start and progression of lung damage. The aim of this study was to investigate the ability of high-resolution computed tomography (HRCT) scoring systems and pulmonary function tests (PFT) to detect changes in lung disease. CF children (n=48) had two HRCT scans in combination with two PFT 2 yrs apart. Their scans were scored using five scoring systems (Castile, Brody, Helbich, Santamaria and Bhalla). "Sensitivity" was defined as the ability to detect disease progression. In this group of children, HRCT scores worsened. PFT remained unchanged or improved. Of the HRCT parameters, mucous plugging and the severity, extent and peripheral extension of bronchiectasis worsened significantly. Relationships between changes in HRCT scores and PFT were weak. Substantial structural lung damage was evident in some children who had normal lung function. These data show that high-resolution computed tomography is more sensitive than pulmonary function tests in the detection of early and progressive lung disease, and suggest that high-resolution computed tomography may be useful in the follow up of cystic fibrosis children and as an outcome measure in studies that aim to reduce lung damage.

摘要

对于囊性纤维化(CF)肺病的有效临床管理而言,密切监测肺损伤的起始和进展非常重要。本研究的目的是调查高分辨率计算机断层扫描(HRCT)评分系统和肺功能测试(PFT)检测肺部疾病变化的能力。48名CF儿童在相隔2年的时间里分别进行了两次HRCT扫描和两次PFT。他们的扫描结果使用五种评分系统(卡斯蒂利亚、布罗迪、赫尔比希、圣玛丽亚和巴哈拉)进行评分。“敏感性”被定义为检测疾病进展的能力。在这组儿童中,HRCT评分恶化。PFT保持不变或有所改善。在HRCT参数中,黏液嵌塞以及支气管扩张的严重程度、范围和外周扩展显著恶化。HRCT评分变化与PFT之间的关系较弱。一些肺功能正常的儿童存在明显的实质性肺损伤。这些数据表明,在检测早期和进展性肺部疾病方面,高分辨率计算机断层扫描比肺功能测试更敏感,并表明高分辨率计算机断层扫描可能有助于囊性纤维化儿童的随访,以及作为旨在减少肺损伤的研究中的一项结局指标。

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