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MCLS 原因不明,多发于4岁以内婴幼儿,是一种急性热性发疹性疾病。1967年在日本首先由川崎氏报道,10余年来在日本发病近1万人。最初以为本病预后良好,近年发现多有心脏损害,且可突然死亡(1～2%);少数患者平安度过急性期,但数年后也可突然死亡。目前认为MCLS 是以冠状动脉为主的全身性中小动脉炎症。突然死亡之剖检病例,全部有冠状动脉血管炎、动脉瘤形成及血栓性闭塞、动脉瘤破裂等。本文对日本MCLS 的研究近况加以探讨(诊断标准可参考其它资料)。
MCLS unexplained, mainly in infants and young children less than 4 years old, is an acute hot rash disease. It was first reported by Kawasaki in Japan in 1967 and nearly 10,000 people in Japan in more than 10 years. Initially that the prognosis of this disease is good, in recent years found that more heart damage, and sudden death (1 ~ 2%); a few patients passed the acute phase of peace, but after a few years can also be a sudden death. MCLS is currently believed to be the main coronary arteries of small artery inflammation. Sudden death of the autopsy cases, all with coronary vasculitis, aneurysm formation and thrombotic occlusion, aneurysm rupture. This article discusses the current situation of Japanese MCLS (diagnostic criteria can refer to other information).