Analysis of the clinical characteristics of patients with Kawasaki disease complicated with cholestasis

BMC Pediatr. 2024 Nov 27;24(1):777. doi: 10.1186/s12887-024-05278-w.

Abstract

Background: To explore the clinical characteristics, related factors, and prognosis of Kawasaki disease (KD) combined with acute febrile cholestasis and improve the understanding of the liver complications of KD to avoid misdiagnosis and missed diagnosis.

Methods: We retrospectively analyzed the demographic, clinical, and laboratory data of 1803 patients with KD between January 2019 and July 2023 in our hospital. Based on the presence of cholestasis, patients were divided into the cholestatic and control groups. Logistic regression analysis was performed for the statistically significant indicators between the two groups to examine the risk factors for KD with coronary artery abnormalities (CAA) and intravenous immunoglobulin (IVIG) unresponsiveness. Additionally, patients with KD and cholestasis were compared with patients with acute febrile cholestasis due to other causes during the same period.

Results: Compared to the control group (n = 1720), patients in the cholestatic group (n = 83) were older, had higher levels of white blood cell count (WBC), C-reactive protein (CRP), D-dimer, N-terminal pro-brain natriuretic peptide (NT-proBNP), a shorter fever duration, and high incidences of IVIG unresponsiveness and CAA. KD was the leading cause of acute febrile cholestasis in children (72.6%). In the multivariate logistic regression analysis, younger age, cholestasis, hypoalbuminemia, and a high NT-proBNP level were risk factors for IVIG unresponsiveness, whereas male sex, longer fever duration before treatment, and high alanine aminotransferase (ALT), and CRP levels were risk factors for CAA.

Conclusion: KD with cholestasis was associated with a higher risk of IVIG unresponsiveness and coronary artery abnormalities. KD was the leading cause of acute febrile cholestasis in children. Attention to the possibility of KD is warranted in children with acute febrile cholestatic jaundice, especially if associated with elevated WBC, CRP, and D-dimer levels, or hypoalbuminemia.

Keywords: Acute febrile cholestasis; Coronary artery abnormalities; Hydrocholecystis; IVIG unresponsiveness; Kawasaki disease.

MeSH terms

  • C-Reactive Protein / analysis
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Cholestasis* / blood
  • Cholestasis* / complications
  • Cholestasis* / diagnosis
  • Cholestasis* / etiology
  • Female
  • Fever* / etiology
  • Humans
  • Immunoglobulins, Intravenous* / therapeutic use
  • Infant
  • Male
  • Mucocutaneous Lymph Node Syndrome* / blood
  • Mucocutaneous Lymph Node Syndrome* / complications
  • Mucocutaneous Lymph Node Syndrome* / diagnosis
  • Natriuretic Peptide, Brain / blood
  • Peptide Fragments / blood
  • Prognosis
  • Retrospective Studies
  • Risk Factors

Substances

  • Immunoglobulins, Intravenous
  • C-Reactive Protein
  • Peptide Fragments
  • pro-brain natriuretic peptide (1-76)
  • Natriuretic Peptide, Brain