The Pathophysiology of Moyamoya Disease: An Update.
The Pathophysiology of Moyamoya Disease: An Update.
复制标题
Moyamoya病的病理生理学：更新。
DOI：
10.5853/jos.2015.01760
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发表时间：
2016-01
影响因子：
8.2
通讯作者：
Kim SK
中科院分区：
文献类型：
--
作者：
Bang OY;Fujimura M;Kim SK
烟雾病（MMD）是一种独特的脑血管疾病，其特征是颅内大动脉进行性狭窄和基底侧支血管网络模糊，称为烟雾血管。由于MMD的病因不明，其诊断是基于特征性的血管造影结果。再血管化技术（例如，旁路手术）用于恢复灌注，并且是MMD的主要治疗。目前尚无预防MMD进展的特异性治疗方法。本文综述了近年来MMD的病理生理学研究进展，包括与疾病发展相关的遗传和循环因素。遗传和环境因素可能以复杂的方式在血管狭窄和异常血管生成的发展中起重要作用。这些因素包括循环内皮/平滑肌祖细胞的相关变化、与血管重塑和血管生成相关的细胞因子以及内皮，如质膜蛋白小窝蛋白。随着对MMD病理生理学的更好理解，针对MMD发病机制的非手术方法可能可用于阻止或减缓这种疾病的进展。可能的策略包括靶向生长因子、视黄酸、小窝蛋白-1和干细胞。
Moyamoya disease (MMD) is a unique cerebrovascular disease characterized by the progressive stenosis of large intracranial arteries and a hazy network of basal collaterals called moyamoya vessels. Because the etiology of MMD is unknown, its diagnosis is based on characteristic angiographic findings. Re-vascularization techniques (e.g., bypass surgery) are used to restore perfusion, and are the primary treatment for MMD. There is no specific treatment to prevent MMD progression. This review summarizes the recent advances in MMD pathophysiology, including the genetic and circulating factors related to disease development. Genetic and environmental factors may play important roles in the development of the vascular stenosis and aberrant angiogenesis in complex ways. These factors include the related changes in circulating endothelial/smooth muscle progenitor cells, cytokines related to vascular remodeling and angiogenesis, and endothelium, such as caveolin which is a plasma membrane protein. With a better understanding of MMD pathophysiology, nonsurgical approaches targeting MMD pathogenesis may be available to stop or slow the progression of this disease. The possible strategies include targeting growth factors, retinoic acid, caveolin-1, and stem cells.