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川崎病:病理生理学、临床表现和治疗。

Kawasaki disease: pathophysiology, clinical manifestations, and management.

机构信息

Division of Pediatric Rheumatology, Departments of Pediatrics, LSU Health Sciences Center and Children's Hospital, New Orleans, LA, USA.

出版信息

Curr Rheumatol Rep. 2014 Jun;16(6):423. doi: 10.1007/s11926-014-0423-x.

Abstract

Kawasaki Disease, a systemic vasculitis of unknown origin with specific predilection for the coronary arteries, is the most common cause of childhood-acquired heart disease in western countries. Despite its world-wide incidence, the pathophysiology of this enigmatic disease is still under investigation. Diagnosis is made on a clinical basis, with supportive laboratory evidence and imaging. Once identified, timely initiation of treatment is imperative in order to quell the inflammatory response and decrease the incidence of long-term sequelae, specifically coronary artery aneurysms. Finally, longitudinal follow-up should be implemented based on risk stratification and individualized to each patient.

摘要

川崎病是一种病因不明的全身性血管炎,特别是对冠状动脉有特殊偏好,是西方国家儿童获得性心脏病的最常见原因。尽管川崎病在全球范围内都有发病,但这种神秘疾病的病理生理学仍在研究中。该疾病的诊断基于临床,同时结合支持性实验室证据和影像学检查。一旦确定,及时开始治疗对于平息炎症反应和降低长期后遗症(特别是冠状动脉瘤)的发生率至关重要。最后,应根据风险分层和个体化制定纵向随访方案。

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