结缔组织变性:掌筋膜变性(Dupuytren病)的机制。

Current molecular biology reports Pub Date : 2016-01-01 Epub Date: 2016-07-14 DOI:10.1007/s40610-016-0045-3
S Karkampouna, M Kreulen, M C Obdeijn, P Kloen, A L Dorjée, F Rivellese, A Chojnowski, I Clark, Marianna Kruithof-de Julio
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引用次数: 12

摘要

Dupuytren病是一种手部结缔组织疾病,引起掌筋膜过度纤维化,伴有手指挛缩和残疾。该病的病因是异质性的,既有遗传因素,也有环境因素。结缔组织被肌成纤维细胞异常浸润,肌成纤维细胞沉积胶原蛋白和其他细胞外基质蛋白。我们描述了Dupuytren病的临床概况以及目前的治疗方案。最近的研究发现,Dupuytren病的分子和细胞参数失调,这可能有助于疾病的发病,并强调了常驻炎症促进纤维化的作用。我们回顾了最近关于非肌成纤维细胞类型(干细胞样细胞)的文献,它们的促炎和促纤维化作用可能解释了异常伤口愈合反应。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Connective Tissue Degeneration: Mechanisms of Palmar Fascia Degeneration (Dupuytren's Disease).

Dupuytren's disease is a connective tissue disorder of the hand causing excessive palmar fascial fibrosis with associated finger contracture and disability. The aetiology of the disease is heterogeneous, with both genetic and environmental components. The connective tissue is abnormally infiltrated by myofibroblasts that deposit collagen and other extracellular matrix proteins. We describe the clinical profile of Dupuytren's disease along with current therapeutic schemes. Recent findings on molecular and cellular parameters that are dysregulated in Dupuytren's disease, which may contribute to the onset of the disease, and the role of resident inflammation promoting fibrosis, are highlighted. We review recent literature focusing on non-myofibroblast cell types (stem cell-like cells), their pro-inflammatory and pro-fibrotic role that may account for abnormal wound healing response.

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