Huang Shen-Wen, Lin Sheng-Chieh, Chen Shih-Yen, Hsieh Kai-Sheng
Division of Allergy, Asthma, and Immunology, Department of Pediatrics, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan.
Department of Primary Care Medicine, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan.
Front Pediatr. 2022 Feb 9;9:738215. doi: 10.3389/fped.2021.738215. eCollection 2021.
Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is a febrile multisystem vasculitis mainly affecting children younger than 5 years. KD typically manifests as skin lesions and in the lymph nodes and oral and conjunctival mucosa. It may induce coronary artery abnormalities, such as aneurysms, but gastrointestinal and hepatobiliary involvement are not common. We reviewed 32 cases of patients with a diagnosis of KD with hepatobiliary involvement between 2000 and 2021 and present the case of a 4-year-old girl who received a diagnosis of KD with combined cholestatic hepatitis and infection. In the 33 cases reviewed, in addition to the classical clinical findings of KD, the most common clinical presentations were jaundice and abdominal pain. Moreover, abnormal laboratory results indicating hyperbilirubinemia, cholestasis, and hepatitis, among other conditions, were noted. Abdominal ultrasonography revealed abnormal findings in more than half children with KD with hepatobiliary involvement. Furthermore, cardiac involvement was noted in a high proportion of the patients. In particular, we noted the case of a 4-year-old girl with a rare presentation of 3-day fever combined with abdominal pain and jaundice. Her levels of aspartate aminotransferase, alanine aminotransferase, total bilirubin, direct bilirubin, alkaline phosphatase, and γ-glutamyl transpeptidase were 489 (15-50) U/L, 253 (5-45) U/L, 4.3 (<1.5) mg/dl, 2.4 (<0.2) mg/dl, 337 (134-315) U/L, and 145 (5-32) U/L, respectively. These results were indicative of cholestatic hepatitis. Furthermore, her serological test results for mycoplasma infection were positive. KD was diagnosed because the patient had high fever for more than 5 days and presented with lymphadenopathy on the left side of neck, a polymorphic skin rash, redness of oral mucosa with strawberry tongue, and nonpurulent conjunctival congestion. After intravenous immunoglobulin injection (IVIG) and acetylsalicylic acid administration, the fever subsided rapidly and clinical manifestations, such as jaundice and abdominal pain, were mitigated. The laboratory parameters gradually returned to within normal ranges. Echocardiography revealed no aneurysm. In conclusion, KD with cholestatic hepatitis should be considered when pediatric patients present with fever combined with abdominal pain and jaundice. Early treatment with IVIG and aspirin is recommended and can effectively relieve cholestatic hepatitis.
川崎病（KD），又称皮肤黏膜淋巴结综合征，是一种发热性多系统血管炎，主要影响5岁以下儿童。KD通常表现为皮肤病变以及淋巴结、口腔和结膜黏膜病变。它可能诱发冠状动脉异常，如动脉瘤，但胃肠道和肝胆受累并不常见。我们回顾了2000年至2021年间32例诊断为KD且有肝胆受累的患者病例，并介绍了一名4岁女孩的病例，该女孩被诊断为KD合并胆汁淤积性肝炎及感染。在回顾的33例病例中，除了KD的典型临床表现外，最常见的临床表现是黄疸和腹痛。此外，还注意到提示高胆红素血症、胆汁淤积和肝炎等异常实验室结果。腹部超声检查显示，超过一半的KD合并肝胆受累儿童有异常发现。此外，高比例患者存在心脏受累情况。特别是，我们注意到一名4岁女孩的罕见病例，其表现为3天发热伴腹痛和黄疸。她的天冬氨酸转氨酶、丙氨酸转氨酶、总胆红素、直接胆红素、碱性磷酸酶和γ-谷氨酰转肽酶水平分别为489（15 - 50）U/L、253（5 - 45）U/L、4.3（<1.5）mg/dl、2.4（<0.2）mg/dl、337（134 - 315）U/L和145（5 - 32）U/L。这些结果提示胆汁淤积性肝炎。此外，她的支原体感染血清学检测结果呈阳性。该患者因高热超过5天，伴有颈部左侧淋巴结肿大、多形性皮疹、口腔黏膜发红伴草莓舌以及非脓性结膜充血而被诊断为KD。静脉注射免疫球蛋白（IVIG）和给予乙酰水杨酸后，发热迅速消退，黄疸和腹痛等临床表现得到缓解。实验室参数逐渐恢复到正常范围。超声心动图显示无动脉瘤。总之，当儿科患者出现发热伴腹痛和黄疸时，应考虑KD合并胆汁淤积性肝炎。建议早期使用IVIG和阿司匹林治疗，可有效缓解胆汁淤积性肝炎。
