小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 多形性皮疹
- 结膜充血
- 粘膜炎
- 外周四肢皮肤变化
- 颈淋巴结增大
- 冠状动脉瘤
- 发热和极易激惹
其他诊断因素
- 渗出性心包炎
- 充血性心力衰竭
- 关节痛或水肿
- 神经系统表现
- 胃肠道临床表现
- 泌尿系统临床表现
- 其他皮肤临床表现
危险因素
- 亚洲裔
- 年龄 3 个月至 4 岁
- 男性
诊断性检查
首要检查
- 全血细胞计数 (FBC）
- 红细胞沉降率 (ESR)
- 血清 CRP
- 超声心动图
需考虑的检查
- 血清肝功能检查
- 尿液分析
- 胸部 X 线检查
- 心电图
- 胆囊超声检查
- 睾丸超声检查
- 腰椎穿刺：
- 磁共振血管造影术
- 心导管检查和血管造影术
- 利钠肽检查
治疗流程
发病 ≤10 日内就诊，或者发病 >10 日后就诊，并伴有持续炎症证据
发病 >10 日后就诊，无持续炎症证据
首次发作后：Z 评分始终＜2；一直无受累
首次发作后：2.0 分≤ Z 评分＜2.5 分；仅扩张
首次发作后：2.5 分≤ Z 评分＜5 分；小型动脉瘤
首次发作后：5 分≤ Z 评分＜10 分（绝对尺寸＜8 mm）；中型动脉瘤
首次发作后：Z 评分≥10 分或绝对尺寸≥8 mm；大型或巨型动脉瘤
撰稿人
作者
Paul Brogan, BSc(Hon), MBChB(Hon), FRCPCH, MSc, PhD
Professor of vasculitis
University College London
London
UK
利益声明
PB is co-chief investigator of the KDCAAP trial, and is an author of several references cited in this topic.
Kirsty McLellan, BMedSci, MBChB, MRCPCH
Specialist Registrar in Paediatric Rheumatology
Great Ormond Street Hospital
London
UK
利益声明
KM declares she has no competing interests.
鸣谢
Dr Paul Brogan and Dr Kirsty McLellan would like to gratefully acknowledge Professor Abraham Gedalia and Dr James Krulisky, previous contributors to this topic.
利益声明
AG declares that he has no competing interests. JK declares that he is a paid consultant for Axia Medical Solutions, a small skincare company from Carlsbad, CA.
同行评议者
Michael Levin, null
Professor of International Child Health
Imperial College London
London
利益声明
ML declares that he has no competing interests
Russell W. Steele, MD
Editor in Chief
Journal of Clinical Pediatrics
Department of Pediatrics
Division of Infectious Diseases
Ochsner Children's Health Center
New Orleans
LA
利益声明
RWS declares that he has no competing interests.
John L. Ey, MD
Clinical Professor of Pediatrics
Department of Pediatrics
Oregon Health Science University
Portland
OR
利益声明
JLE declares that he has no competing interests.
David Burgner, BSc(Hons), MBChB, MRCP, MRCPCH, FRACP, DTMH, PhD
Principal Research Fellow
Murdoch Childrens Research Institute
The Royal Children’s Hospital
Victoria
Australia
利益声明
DB has received competitive research funding from the National Heart Foundation Australia and from the Agency for Science, Technology and Research of the Singapore Government. He is co-inventor on a patent related to diagnostics submitted through the Genome Institute of Singapore.
鉴别诊断
- 葡萄球菌或链球菌感染
- 系统性青少年特发性关节炎（系统性 JIA）
- 猩红热
更多 鉴别诊断指南
- 川崎病管理指南
- 基于欧洲共识的川崎病诊断和治疗建议——SHARE 倡议
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