医学
肌成纤维细胞
细胞外基质
转录组
特发性肺纤维化
纤维化
病态的
病理
肺纤维化
呼吸系统
间质性肺病
呼吸衰竭
肺功能测试
组织重塑
细胞外
呼吸道疾病
肺
发病机制
寻常性间质性肺炎
肺纤维化
作者
A. Justet,Jonas C. Schupp
出处
期刊:The European respiratory journal
[European Respiratory Society]
日期:2025-10-01
卷期号:66 (4): 2501466-2501466
标识
DOI:10.1183/13993003.01466-2025
摘要
Extract Fibrosis is a pathological wound-healing response characterised by the excessive deposition of extracellular matrix components, ultimately disrupting the architecture and function of affected organs [1]. Idiopathic pulmonary fibrosis (IPF), the most severe form of idiopathic interstitial lung disease, exemplifies this process. IPF is a progressive and ultimately fatal disorder marked by irreversible remodelling of the lung parenchyma, leading to impaired gas exchange and respiratory failure [2].
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