川崎病

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最后审阅： 10 五月 2023
最后更新： 14 十二月 2022

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
更多 关键诊断因素

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
其他诊断因素

危险因素

  • 亚洲裔
  • 年龄 3 个月至 4 岁
  • 男性
更多 危险因素
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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
更多 首要检查

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
更多 需考虑的检查
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治疗流程

急症处理

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

持续性治疗

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

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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.

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