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@article{Dean:2020:10.1136/thoraxjnl-2020-214752,
author = {Dean, C and Taylor, B and Rice, A and Nicholson, J and Hind, M},
doi = {10.1136/thoraxjnl-2020-214752},
journal = {Thorax},
pages = {1001--1003},
title = {Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations},
url = {http://dx.doi.org/10.1136/thoraxjnl-2020-214752},
volume = {75},
year = {2020}
}

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TY  - JOUR
AB  - Congenital pulmonary airway malformations (CPAMs) are rare lung abnormalities that result in cyst formation and are associated with respiratory distress in infants and malignant potential in adults. The pathogenesis of CPAMs remains unknown but data suggest disruption of the normal proximo-distal programme of airway branching and differentiation. Here, we demonstrate that adult human CPAM are lined with epithelium that retains SOX-2 and thyroid transcription factor-1 immunohistochemical markers, characteristic of the developing lung. However, RALDH-1, another key marker, is absent. This suggests a more complex aetiology for CPAM than complete focal arrest of lung development and may provide insight to the associated risk of malignancy.
AU  - Dean,C
AU  - Taylor,B
AU  - Rice,A
AU  - Nicholson,J
AU  - Hind,M
DO  - 10.1136/thoraxjnl-2020-214752
EP  - 1003
PY  - 2020///
SN  - 0040-6376
SP  - 1001
TI  - Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations
T2  - Thorax
UR  - http://dx.doi.org/10.1136/thoraxjnl-2020-214752
UR  - https://thorax.bmj.com/content/75/11/1001
UR  - http://hdl.handle.net/10044/1/81289
VL  - 75
ER  -

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