以胆汁淤积性黄疸为首发症状的川崎病临床特点

肝脏 ›› 2025, Vol. 30 ›› Issue (7): 1005-1009.

以胆汁淤积性黄疸为首发症状的川崎病临床特点

张懿, 袁娟, 杨亚荣, 朱欣欣

710003 陕西西安西安市儿童医院感染性疾病门诊

收稿日期:2025-02-23 出版日期:2025-07-31 发布日期:2025-08-11
通讯作者: 朱欣欣, Email:why1986818@163.com

Clinical characteristics of Kawasaki disease with cholestatic jaundice as the initial symptom

ZHANG Yi, YUAN Juan, YANG Ya-rong, ZHU Xin-xin

Department of Infection, Xi’an Children’s Hospital, the Affiliated Children’s Hospital of Xi’an Jiaotong University, Xi’an 710003, China

Received:2025-02-23 Online:2025-07-31 Published:2025-08-11
Contact: ZHU Xin-xin, Email:why1986818@163.com

摘要/Abstract

摘要： 目的探讨以胆汁淤积症为初始症状的川崎病(KD)的临床特征,提高对KD的认识。方法回顾性分析2018年1月1日至2024年12月31日于西安市儿童医院收治的4 381例KD患者。收集以胆汁淤积性黄疸为首发症状的患者的人口统计学、临床和实验室资料。根据患者对丙种免疫球蛋白(IVIG)的反应,将患者分为IVIG无反应KD组和IVIG反应KD组,以确定IVIG耐药的潜在危险因素。结果 4 381例KD患者中,以胆汁淤积性黄疸为首发症状的有29例(0.66%),男女比例为1.63∶1。发病年龄中位数为4.33(3.17,6.17)岁,发病第3 (3,3.5)d出现黄疸。入院时,总胆红素(TBil)和直接胆红素(DBil)中位数分别为82.75 (72.70,102.10)μmol/L和63.5 (45.65,71.55)μmol/L。所有患者在给予IVIG前均行心脏及肝胆超声检查,其中10例出现冠状动脉异常,12例有肝胆异常。从发病到KD诊断的中位时间为6 (5,6)d,诊断后立即开始IVIG治疗,其中12例为IVIG无反应KD。以胆汁淤积性黄疸为首发症状的KD患者IVIG耐药率显著高于其他患者(χ² =62.01, P<0.001)。除1例外,其余28例出院前肝功能指标均恢复正常。IVIG无反应组与IVIG反应组相比,IVIG无反应组患者接受IVIG治疗时间较早(Z=-1.568, P=0.049)。结论胆汁淤积性黄疸患儿需要注意KD发生的可能性,且以胆汁淤积为首发症状的KD更易出现IVIG无反应。

关键词: 川崎病, 胆汁淤积性黄疸, IVIG耐药

Abstract: Objective To explore the clinical characteristics of Kawasaki disease (KD) with cholestatic jaundice as the initial symptom, to enhance awareness and understanding of KD. Methods A retrospective analysis was conducted on 4,381 KD patients admitted between January 1, 2018 and December 31, 2024. Demographic, clinical, and laboratory data were collected from patients with febrile cholestatic jaundice as their first symptom. Patients were categorized into IVIG-resistant and IVIG-non-resistant groups based on their response to IVIG to identify the potential risk factors for IVIG resistance. Results Among 4,381 KD cases, 29 (0.66%) presented with cholestatic jaundice as the initial symptom. The median age of onset was 4.33 (3.17, 6.17) years, and jaundice appeared on the 3rd (3,3.5) day of the illness. At admission, the median total bilirubin (TBil) and direct bilirubin (DBil) levels were 82.75 (72.70, 102.10) μmol/L and 63.5 (45.65, 71.55) μmol/L, respectively. All patients underwent cardiac and hepatobiliary ultrasound before IVIG administration, revealing coronary artery abnormalities (CAA) in 10 cases and hepatobiliary abnormalities in 12 cases. The median time from onset to KD diagnosis was 6 (5,6) days, and IVIG treatment was initiated immediately after diagnosis, with 12 cases showing IVIG resistance. KD with cholestatic jaundice as the initial symptom exhibited a significantly higher IVIG resistance rate (χ²=62.01, P=0.00). Comparing the IVIG-resistant and non-resistant groups, those in the IVIG-resistant group received IVIG treatment earlier (Z=-1.568, P=0.049). Conclusion Clinicians should be vigilant for KD cases presenting with cholestatic jaundice, and this subset is more likely to develop IVIG resistance.

Key words: Kawasaki disease, Cholestatic jaundice, IVIG resistance

张懿, 袁娟, 杨亚荣, 朱欣欣. 以胆汁淤积性黄疸为首发症状的川崎病临床特点[J]. 肝脏, 2025, 30(7): 1005-1009.

ZHANG Yi, YUAN Juan, YANG Ya-rong, ZHU Xin-xin. Clinical characteristics of Kawasaki disease with cholestatic jaundice as the initial symptom[J]. Chinese Hepatolgy, 2025, 30(7): 1005-1009.

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