Inserm, UMR-S U938, Paris, F-75012 France.
Paediatr Respir Rev. 2011 Dec;12(4):216-22. doi: 10.1016/j.prrv.2011.04.003. Epub 2011 May 18.
Interstitial lung diseases (ILDs) in children represent a heterogeneous group of respiratory disorders characterized by derangements of the alveolar walls. The key pathologic feature of ILDs is the altered repair of the alveolar surface after injury with a marked disruption in the integrity of the epithelium and, consequently, a dysregulated communication between epithelial and mesenchymal pulmonary components. Concomitant to the loss of cell-cell contact, epithelial cells undergo a process called epithelial to mesenchymal transition and acquire a mesenchymal identity. Among the factors involved in disease progression, transforming growth factor-β has been identified as a master switch in the induction of fibrosis. This article reviews recent advances in the understanding of the mechanisms involved in the pathogenesis of ILDs, and provides information on their adaptation in the context of lung growth.
儿童间质性肺疾病（ILDs）代表了一组异质性的呼吸系统疾病，其特征是肺泡壁的紊乱。ILDs 的主要病理特征是肺泡表面在损伤后的异常修复，上皮完整性明显中断，因此上皮和间充质肺成分之间的通讯失调。伴随着细胞-细胞接触的丧失，上皮细胞经历一个称为上皮-间充质转化的过程，并获得间充质特征。在参与疾病进展的因素中，转化生长因子-β已被确定为纤维化诱导的主开关。本文综述了ILDs 发病机制中相关机制的最新研究进展，并提供了关于其在肺生长背景下适应性的信息。
