Abstract

Cystic fibrosis is an autosomal recessive disorder, and the product of the gene results in an epithelial cell membrane chloride channel defect [2]. The abnormally low water content of mucus leads to blockage of bronchioles and bronchi resulting in infection and inflammation which progresses to bronchiectasis. The clinical course of bronchiectasis is interspersed with episodic acute exacerbations of pulmonary infection leading to peribronchial fibrosis and progressive loss of pulmonary function and respiratory failure. To treat this, double lung transplantation has been successful in cystic fibrosis patients with end stage lung disease [3]. On high resolution CT scans, cystic fibrosis is manifest as bronchiectasis and a mosaic pattern of lung attenuation which reflects small airways disease and air trapping. Expiratory high resolution CT scans accentuate the mosaic pattern of lung attenuation caused by air trapping [4,5], indeed, the expiratory high resolution CT scan is necessary to help to differentiate among different causes of a mosaic pattern of lung attenuation [4]. Air trapping is accentuated by expiration because the areas of decreased attenuation on the CT scan remain unchanged in volume and attenuation, whereas the uninvolved lung shows a homogenous increase in attenuation [5,6]. MinIP images accentuate the differences in lung attenuation due to the inherent high-contrast resolution. As expiration accentuates air trapping, expiratory MinlP images are potentially very useful to demonstrate the mosaic pattern of lung attenuation caused by small airways disease. Air trapping has been demonstrated following forced expiration of subjects with normal pulmonary function [7,8]. Up to 25% of the cross-sectional area of one section of lung has been shown to demonstrate air-trapping in normal subjects [7], in our patient approximately 70% and 60% of the cross-sectional area of the

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