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Title
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Morphometric analysis of explant lungs in cystic fibrosis
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Author
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Abstract
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| Rationale: After repeated cycles of lung infection and inflammation, patients with cystic fibrosis (CF) evolve to respiratory insufficiency. Although histology and imaging have provided descriptive information, a thorough morphometric analysis of end-stage CF lung disease is lacking. Objectives: To quantify the involvement of small and large airways in end-stage CF. Methods: Multidetector computed tomography (MDCT) and micro-CT were applied to 11 air-inflated CF explanted lungs and 7 control lungs to measure, count, and describe the airway and parenchymal abnormalities in end-stage CF lungs. Selected abnormalities were further investigated with thin section histology. Measurements and Main Results: On MDCT, CF explanted lungs showed an increased median (interquartile range) number (631 [511-710] vs. 344 [277-349]; P = 0.003) and size of visible airways (cumulative airway diameter 217 cm [209-250] vs. 91 cm [80-105]; P < 0.001) compared with controls. Airway obstruction was seen, starting from generation 6 and increasing to 40 to 50% of airways from generation 9 onward. Micro-CT showed that the total number of terminal bronchioles was decreased (2.9/ml [2.6-4.4] vs. 5.3/ml [4.8-5.7]; P < 0.001); 49% were obstructed, and the cross-sectional area of the open terminal bronchioles was reduced (0.093 mm(2) [0.084- 0.123] vs. 0.179 mm(2) [0.140-0.196]; P < 0.001). On micro CT, 41% of the obstructed airways reopened more distally. This remodeling was confirmed on histological analysis. Parenchymal changes were also seen, mostly in a patchy and peribronchiolar distribution. Conclusions: Extensive changes of dilatation and obstruction in nearly all airway generations were observed in end-stage CF lung disease. |
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Language
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English
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Source (journal)
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American journal of respiratory and critical care medicine. - New York, 1994, currens
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Publication
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New York
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2016
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ISSN
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1073-449X
[print]
1535-4970
[online]
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DOI
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10.1164/RCCM.201507-1281OC
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Volume/pages
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193
:5
(2016)
, p. 516-526
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ISI
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000371753300012
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Pubmed ID
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26551917
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Full text (Publisher's DOI)
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