Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations.
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.
['Taylor|Bethany|B|', 'Rice|Alexandra|A|', 'Nicholson|Andrew G|AG|', 'Hind|Matthew|M|', 'Dean|Charlotte H|CH|0000-0002-8846-5472']
D000328:Adult D000080924:Aldehyde Dehydrogenase 1 Family D015415:Biomarkers D002454:Cell Differentiation D004268:DNA-Binding Proteins D006801:Humans D066298:In Vitro Techniques D008168:Lung D020545:Respiratory Mucosa D015619:Respiratory System Abnormalities D050697:Retinal Dehydrogenase D055748:SOXB1 Transcription Factors D014157:Transcription Factors
TTF-1 airway epithelium lung cancer