Morphometric analysis of explant lungs in cystic fibrosis

M Boon, SE Verleden, B Bosch… - American journal of …, 2016 - atsjournals.org
M Boon, SE Verleden, B Bosch, EJ Lammertyn, JE McDonough, C Mai, J Verschakelen
American journal of respiratory and critical care medicine, 2016atsjournals.org
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 …
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 mm2 [0.084–0.123] vs. 0.179 mm2 [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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