Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease A Scientific Statement for Health Professionals From the American Heart Association
Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease A Scientific Statement for Health Professionals From the American Heart Association
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DOI：
10.1161/cir.0000000000000484
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发表时间：
2017-04-25
期刊：
影响因子：
37.8
通讯作者：
Pahl, Elfriede
中科院分区：
文献类型：
--
作者：
McCrindle, Brian W.;Rowley, Anne H.;Pahl, Elfriede
背景：川崎是一种儿童急性血管炎，约25%的未治疗病例会导致冠状动脉瘤。它已被报道在世界范围内，是获得性心脏病的主要原因，在发达国家的儿童informations.METHODS和结果：修改以前的美国心脏协会的指导方针，一个多学科的专家写作小组召开审查和评估现有的证据和实践为基础的意见，以及提供最新的建议，诊断，治疗的急性疾病，和长期管理。虽然原因仍然未知，但讨论部分强调了对流行病学，遗传学，发病机制，病理学，自然史和长期结果的新见解。及时的诊断是必不可少的，更新的算法定义了补充信息，用于在经典临床标准不完整时辅助诊断。虽然静脉注射免疫球蛋白是主要的初始治疗，在选定的患者中的作用进行了讨论。大约10%至20%的患者对初始静脉注射免疫球蛋白没有反应，并提供了额外治疗的建议。对进展中的冠状动脉异常进行仔细的初始管理是至关重要的，需要增加评估频率和血栓预防措施的升级。长期管理的风险分层主要基于最大冠状动脉管腔尺寸，标准化为Z评分，并根据既往和当前受累情况进行校准。动脉瘤患者需要终身和不间断的心脏病follow-up.CONCLUSIONS：这些建议提供了最新的和最好的循证指导医疗服务提供者诊断和管理川崎病，但临床决策应个性化的具体情况下，患者。
BACKGROUND: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in approximate to 25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.METHODS AND RESULTS: To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.CONCLUSIONS: These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.