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成人烟雾病和综合征:现状和未来方向:美国心脏协会/美国中风协会的科学声明。

Adult Moyamoya Disease and Syndrome: Current Perspectives and Future Directions: A Scientific Statement From the American Heart Association/American Stroke Association.

出版信息

Stroke. 2023 Oct;54(10):e465-e479. doi: 10.1161/STR.0000000000000443. Epub 2023 Aug 23.

Abstract

Adult moyamoya disease and syndrome are rare disorders with significant morbidity and mortality. A writing group of experts was selected to conduct a literature search, summarize the current knowledge on the topic, and provide a road map for future investigation. The document presents an update in the definitions of moyamoya disease and syndrome, modern methods for diagnosis, and updated information on pathophysiology, epidemiology, and both medical and surgical treatment. Despite recent advancements, there are still many unresolved questions about moyamoya disease and syndrome, including lack of unified diagnostic criteria, reliable biomarkers, better understanding of the underlying pathophysiology, and stronger evidence for treatment guidelines. To advance progress in this area, it is crucial to acknowledge the limitations and weaknesses of current studies and explore new approaches, which are outlined in this scientific statement for future research strategies.

摘要

成人烟雾病和烟雾综合征是罕见疾病,具有较高的发病率和死亡率。我们选择了一组专家撰写小组进行文献检索,总结了该主题的现有知识,并为未来的研究提供了路线图。本文更新了烟雾病和烟雾综合征的定义、现代诊断方法,以及病理生理学、流行病学、内科和外科治疗的最新信息。尽管最近取得了一些进展,但烟雾病和烟雾综合征仍有许多悬而未决的问题,包括缺乏统一的诊断标准、可靠的生物标志物、对潜在病理生理学的更好理解,以及治疗指南的更强证据。为了推动该领域的进展,必须认识到当前研究的局限性和弱点,并探讨新的方法,本科学声明为未来的研究策略勾勒出了这些方法。

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