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囊性纤维化。概述。

Cystic fibrosis. An overview.

作者信息

Ruzal-Shapiro C

机构信息

Department of Radiology, Columbia University, College of Physicians and Surgeons, New York, New York, USA.

出版信息

Radiol Clin North Am. 1998 Jan;36(1):143-61. doi: 10.1016/s0033-8389(05)70011-7.

DOI:10.1016/s0033-8389(05)70011-7
PMID:9465872
Abstract

A single gene defect in cystic fibrosis results in a spectrum of clinical presentations. Though expression is variable, the radiographic appearance reflects the underlying pathophysiology. Abnormalities of the small airways result in air-trapping; bronchiectasis and mucus plugging reflect large airway disease. These radiographic findings can be assessed and followed using varying scoring systems.

摘要

囊性纤维化中的单个基因缺陷会导致一系列临床表现。尽管表达存在差异,但影像学表现反映了潜在的病理生理学。小气道异常导致气体潴留;支气管扩张和黏液阻塞反映大气道疾病。这些影像学表现可以使用不同的评分系统进行评估和随访。

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Non-invasive pulmonary perfusion assessment in young patients with cystic fibrosis using an arterial spin labeling MR technique at 1.5 T.
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Comparative evaluation of chest radiography, low-field MRI, the Shwachman-Kulczycki score and pulmonary function tests in patients with cystic fibrosis.囊性纤维化患者胸部X线摄影、低场MRI、Shwachman-Kulczycki评分及肺功能测试的比较评估
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