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Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations
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  • Published on:
    Congenital pulmonary airway malformations: what's new?
    • Isabelle Ruchonnet-Métrailler, Pediatric Pulmonologist Hôpitaux Universitaire de Genève, Geneva, Switzerland
    • Other Contributors:
      • Guillaume Lezmi, Pediatric Pulmonologist
      • Christophe Delacourt, Pediatric pulmonologist

    We have read with interest the article by Taylor et al. concerning "the mechanism of lung development in the etiology of congenital malformations of the pulmonary airways in adults". The authors discussed the etiology of congenital malformations of the pulmonary airways, suggesting a partial modification of lung development with a potential risk of malignancy.

    Although we generally agree with their assessment, there are some weaknesses in their work that we would like to highlight as well as some points on which we would like to propose an alternative point of view. Different transcription factors known to be involved in lung development have already been studied in CPAM. Two of them, SOX2 and SOX9 are described as important in the spatiotemporal branching development since the pseudoglandular stage [1, 2]. In CPAM, SOX2 is present in both CPAM types (1 and 2), but their expression differs between them [3]. In addition, previously published papers have shown persistent SOX2 expression in healthy lung, which is not the case in this paper. Unfortunately, Talyor et al present "adult" samples and not adjacent healthy. However, this is not sufficient to explain these differences and classical tissues from children should have been included to demonstrate this point. Moreover, a difference in the cells forming the two types of CPAM has already been described by immunohistochemistry and proteomic results. Nevertheless these points are not addressed in t...

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    Conflict of Interest:
    None declared.