High resolution computed tomography in adult cystic fibrosis patients with mild lung disease

doi:10.1016/S0009-9260(05)80220-X

Clinical Radiology

Volume 44, Issue 1, July 1991, Pages 20-22

https://doi.org/10.1016/S0009-9260(05)80220-X Get rights and content

Chest radiography and high resolution computed tomography (HRCT) were used to evaluate the degree of lung involvement in 38 adult cystic fibrosis patients with unusually mild pulmonary disease. Abnormal features were present on the chest radiograph and/or high resolution CT scans of 35 patients, while in three patients both investigations were normal. Thickening of the wall of proximal right upper lobe bronchi was the earliest abnormal feature on HRCT. The commonest abnormal feature in these patients, including those whose chest radiograph was normal, was mild, uniform dilatation of proximal bronchi. The lumen dilatation was always less marked than the degree of thickening. Ten of the patients were first diagnosed in adult life. Although HRCT scans were normal in two of these patients, there were features suggestive of early pulmonary disease in the remaining eight.

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The right upper lobe was most commonly affected by bronchiectasis, although there was no overall upper lobe predominance, due in part to a relatively low frequency of abnormalities detected in the left upper lobe. This could explain observations in previous studies using chest CT6-9 and chest radiography14 that report an upper lobe predominance for structural changes. Although CF lung disease is traditionally considered to affect the upper lobes more than the lower lobes, we failed to demonstrate an overall upper lobe predominance for any structural lung disease component in the young children with early disease, but the lobe most commonly affected by bronchiectasis was the right upper lobe.
To examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed tomography (CT).
This cross-sectional study included 62 children aged 1-6 years with volume-controlled volumetric chest CT scans performed under general anesthesia as part of an early surveillance program. Each lobe was scored for presence and extent of bronchiectasis, mucus plugging, and air trapping using a semiquantitative score. The topographic distribution of structural abnormalities was evaluated by comparing the presence and extent of abnormalities in different lung regions and examining relationships between components.
Although bronchiectasis was most common in the right upper lobe, overall changes in lung structure were not more common or more extensive in the upper lobes. Rather, bronchiectasis was more common in the right lung (right lung 0.95, left lung 0.68, P = .003), and mucus plugging (upper 0.41, middle 0.41, lower 0.72, P = .028) and air trapping (upper 0.79, middle 0.48, lower 0.96, P < .001) were more common in the lower lobes. The extents of bronchiectasis (P < .001) and air trapping (P = .011) were greater in the right lung. Scans with bronchiectasis were also more likely to have coexisting mucus plugging (P = .008) and air trapping (P < .001).
Early structural lung disease is heterogeneously distributed in the lung. Quantitative scoring tools for studies using chest CT as an end point, and mechanistic studies that seek to better understand the pathogenesis of early cystic fibrosis lung disease, should take account of this differential topographic expression of disease early in life.
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Third, there has always been the perplexing question in the natural history of CF lung disease whereby the upper lobes develop disease before the lower lobes (54). Because of gravitational effects, the upper lobes are ventilated less (by ∼50%) than the lower lobes (55). The reduced upper lobe tidal volume would reduce airflow-induced shear on airway epithelia, leading to less local nucleotide release.
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2004, Journal of Pediatrics
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Original PaperHigh resolution computed tomography in adult cystic fibrosis patients with mild lung disease

Clinical Radiology

Clinical Radiology

Pulmonary cystic fibrosis in the adult: early and late radiological findings with pathological correlation

American Journal of Roentgenology

High resolution computed tomography in pulmonary cystic fibrosis

British Journal of Radiology

Original Paper
High resolution computed tomography in adult cystic fibrosis patients with mild lung disease