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川崎病:诊断、管理及长期影响

Kawasaki disease: diagnosis, management, and long-term implications.

作者信息

Satou Gary M, Giamelli Joseph, Gewitz Michael H

机构信息

Section of Pediatric Cardiology, Maria Fareri Children's Hospital, Westchester Medical Center/New York Medical College, Valhalla, New York 10595, USA.

出版信息

Cardiol Rev. 2007 Jul-Aug;15(4):163-9. doi: 10.1097/CRD.0b013e31802ea93f.

DOI:10.1097/CRD.0b013e31802ea93f
PMID:17575479
Abstract

Kawasaki disease (KD) is an acute inflammatory vasculitis of childhood which was initially described more than 4 decades ago, yet the specific etiology remains unknown. It has become the most common cause of acquired cardiovascular disease in children in the United States. Advances in clinical therapies have reduced, but not eliminated, the incidence of coronary artery abnormalities in affected children. Pathophysiology seems to include an intense elaboration of cytokines, endothelin, and other vasoactive mediators resulting in the development of vascular endothelial changes that may leave a permanent impact on vascular integrity. Treatment with intravenous immune globulin and aspirin remains the primary management strategy and steroid therapy remains contoversial. In severe circumstances, coronary reperfusion strategies are required, and coronary artery surgery in children with KD has been required, albeit infrequently. KD may be a harbinger for early onset coronary artery disease in adults. Recently developed AHA recommendations have amended diagnostic strategies and indicated a stratified approach to the long-term follow up of this enigmatic yet widespread disease.

摘要

川崎病(KD)是一种儿童急性炎症性血管炎,最初在40多年前被描述,但其具体病因仍不清楚。它已成为美国儿童后天性心血管疾病的最常见病因。临床治疗的进展降低了(但并未消除)患病儿童冠状动脉异常的发生率。病理生理学似乎包括细胞因子、内皮素和其他血管活性介质的大量释放,导致血管内皮变化的发展,这可能对血管完整性产生永久性影响。静脉注射免疫球蛋白和阿司匹林治疗仍然是主要的治疗策略,而类固醇治疗仍存在争议。在严重情况下,需要采取冠状动脉再灌注策略,并且尽管很少见,但KD患儿也需要进行冠状动脉手术。KD可能是成人早发性冠状动脉疾病的先兆。最近制定的美国心脏协会(AHA)建议修订了诊断策略,并指出了对这种神秘但普遍存在的疾病进行长期随访的分层方法。

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Kawasaki disease: diagnosis, management, and long-term implications.川崎病:诊断、管理及长期影响
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