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Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations.

ABSTRACT: 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.

SUBMITTER: Taylor B

PROVIDER: S-EPMC7569368 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations.

Taylor Bethany B Rice Alexandra A Nicholson Andrew G AG Hind Matthew M Dean Charlotte H CH

Thorax 20200730 11

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, ...[more]

PMID: 32732323

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