Cholestasis as a complication in Kawasaki disease: an atypical clinical presentation.

PURPOSE: Cholestasis is an uncommon complication in Kawasaki disease (KD) that impacts the digestive system. This study aimed to examine the clinical characteristics and associated risk factors of KD complicated by cholestasis. METHODS: Among the 3026 KD patients admitted to Yuying Children's Hospital, affiliated with Wenzhou Medical University, from January 1, 2008, to December 31, 2020, all 72 children with cholestasis were included in the study. We performed a comprehensive analysis of the clinical characteristics, prognosis, and potential risk factors associated with KD patients presenting with cholestasis. RESULTS: All 72 patients (median age: 34.1 months; 52.8% male) met the diagnostic criteria for cholestatic hepatitis. The mean duration of hospitalization was 10 days. Clinically, the incidence of limb edema and cervical lymphadenitis was relatively low. Pre-treatment assessments of liver function revealed significantly elevated levels of alanine aminotransferase, aspartate aminotransferase, and γ-glutamyl transpeptidase, with median values of 227 U/L, 102 U/L, and 220 U/L, respectively. Abdominal ultrasonography indicated an increased incidence of cholecystitis (12.5%) and hepatosplenomegaly (32.9%), in the absence of gallstones. Additionally, patients demonstrated an increased likelihood of systemic involvement, including pancreatitis and aseptic pyuria, alongside significantly elevated inflammatory markers such as C-reactive protein (CRP), white blood cell count (WBC), and neutrophil levels. All patients received treatment with intravenous immunoglobulin (IVIG) and aspirin, leading to the normalization of liver function indicators within one week. In this study, 25.0% of the patients demonstrated resistance to IVIG therapy, with 2 cases experiencing acute pancreatitis and shock. Furthermore, 25 patients developed coronary artery lesions, which resolved over a 32-month follow-up period as assessed by cardiac ultrasound. CONCLUSION: When KD is complicated by cholestasis, it impacts the liver and bile ducts, leading to liver damage, cholecystitis, and jaundice, while also intensifying systemic inflammatory responses and increasing the likelihood of additional systemic involvements. Despite these complications, the prognosis is generally favorable; however, there is an associated elevated risk of resistance to IVIG therapy.