RT Journal Article
SR Electronic
T1 Quantitative evaluation of CF airways using HRCT, µCT and histology
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 3447
VO 44
IS Suppl 58
A1 Mieke Boon
A1 Barbara Bosch
A1 Elise Lammertyn
A1 Stijn Verleden
A1 Pieter Goeminne
A1 Bart Vanaudenaerde
A1 Dirk Van Raemdonck
A1 Erik Verbeken
A1 Geert Verleden
A1 James Hogg
A1 Johny Verschakelen
A1 Lieven Dupont
A1 Kris De Boeck
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/3447.abstract
AB Aim: To study large and small airway abnormalities in end-stage cystic fibrosis (CF).Methods: 10 CF explant lungs and 6 control (C) lungs were inflated to TLC, frozen at -80°C and scanned using high resolution computed tomography (HRCT) (1 mm slices, max resolution 0.6 mm). Lung tissue cores (1.4 cm x 2 cm) were randomly excised, processed and scanned at a resolution of 8.4 µm using a MicroComputerized Tomography (µCT) device (Skyscan 1172). Using Osirix 4.1 and as previously described (Verleden et al, AJRCCM 2014), we manually assessed the number and size of (visible) open and obstructed airways per generation on HRCT, and the number and size of terminal bronchioles (TB) on µCT. From selected regions in the cores histological sections were sliced.Results: Compared to C, CF had more visible airways on HRCT (567 vs 310; p 0.007). Airway dilatation started from generation 6 (total cumulative airway diameter 203 vs 78cm, p 0.0005). From generation 6 on, around 40% of airways per generation were obstructed. The number of visible airways correlated with the Brody bronchiectasis subscore (R 0.64; p 0.045). In CF versus C, open TB were reduced in number (2.9 vs 5.7/ml; p 0.002) and size (212 vs 363 µm, p 0.004). Closed TB (46%) were fibrotic in 34 or collapsed in 12 % resp. Histology showed narrowed small airways distal to bronchiectasis with disappearance of the lumen and replacement of the airway wall by scar tissue. In 1/5 of studied airways, more distal reorganization into airways with open lumen occurred.Conclusion: End-stage CF causes airway obstruction and dilatation from airway generation 6 on. Distally there is decrease in number and narrowing of TB. Remodeling of small airways was seen on µCT and histology.