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Until now, the cellular and molecular mechanisms of the development of lung defects remain a poorly studied area of pulmonology. In the occurrence of anomalies in the airways of the lungs, a change in the expression of proteins that control early lung morphogenesis in normal conditions (proteins FGF, TGF, SHH, WNT) was established. Along with this, bronchial markers and markers of type 2 alveolocytes play a certain role in the occurrence of lung defects. A number of congenital malformations are caused by improper formation of the airways, which may be associated with the influence of various soluble factors, receptors, transcription factors and microRNAs. The possible role of the pulmonary neuroendocrine system (apudocytes and neuroepithelial bodies) in the pathogenesis and pathobiology of childhood lung diseases, including congenital lung diseases, is discussed.

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