1 Department of Pharmacology, Rush University, Chicago, Illinois, USA.
Pulm Circ. 2013 Sep;3(3):478-98. doi: 10.1086/674439. Epub 2013 Dec 5.
Abstract Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are severe syndromes resulting from the diffuse damage of the pulmonary parenchyma. ALI and ARDS are induced by a plethora of local or systemic insults, leading to the activation of multiple pathways responsible for injury, resolution, and repair or scarring of the lungs. Despite the large efforts aimed at exploring the roles of different pathways in humans and animal models and the great strides made in understanding the pathogenesis of ALI/ARDS, the only viable treatment options are still dependent on ventilator and cardiovascular support. Investigation of the pathophysiological mechanisms responsible for initiation and resolution or advancement toward lung scarring in ALI/ARDS animal models led to a better understanding of the disease's complexity and helped in elucidating the links between ALI and systemic multiorgan failure. Although animal models of ALI/ARDS have pointed out a variety of new ideas for study, there are still limited data regarding the initiating factors, the critical steps in the progression of the disease, and the central mechanisms dictating its resolution or progression to lung scarring. Recent studies link deficiency of intersectin-1s (ITSN-1s), a prosurvival protein of lung endothelial cells, to endothelial barrier dysfunction and pulmonary edema as well as to the repair/recovery from ALI. This review discusses the effects of ITSN-1s deficiency on pulmonary endothelium and its significance in the pathology of ALI/ARDS.
摘要急性肺损伤 (ALI) 和急性呼吸窘迫综合征 (ARDS) 是由肺实质弥漫性损伤引起的严重综合征。ALI 和 ARDS 由大量局部或全身损伤引起,导致多个通路的激活,这些通路负责肺的损伤、修复和修复或瘢痕形成。尽管人们在探索不同通路在人类和动物模型中的作用方面付出了巨大努力,并在理解 ALI/ARDS 的发病机制方面取得了重大进展,但唯一可行的治疗选择仍然依赖于呼吸机和心血管支持。对 ALI/ARDS 动物模型中负责起始和修复或进展为肺瘢痕形成的病理生理机制的研究,加深了对该疾病复杂性的理解,并阐明了 ALI 与全身多器官衰竭之间的联系。尽管 ALI/ARDS 的动物模型提出了各种新的研究思路,但关于起始因素、疾病进展的关键步骤以及决定其修复或进展为肺瘢痕形成的核心机制的相关数据仍然有限。最近的研究将 intersectin-1s (ITSN-1s) 的缺乏与肺内皮细胞的生存蛋白联系起来,其与内皮屏障功能障碍和肺水肿有关,也与 ALI 的修复和恢复有关。这篇综述讨论了 ITSN-1s 缺乏对肺内皮细胞的影响及其在 ALI/ARDS 病理中的意义。