To the Editor: The diagnosis of Kawasaki disease (KD) is based on clinical criteria and there exist no specific diagnostic laboratory tests [1]. Although KD is the common acute febrile disease in children, especially in Asia [2,3,4], seldom is the cholestasis reported as the initial feature of KD.

We report 7 children who presented atypical onset of KD with acute febrile cholestatic jaundice as initial feature. The average age was 3 y. Jaundice occurred within 3 d, and 2 cases occurred on the first day. The median of total bilirubin, direct bilirubin, aspartate aminotransferase, gamma glutamyl transferase, and the mean of alanine aminotransferase were 4.84 mg/dL, 3.87 mg/dL, 72 U/L, 146.53 μmol/L, 171.7 U/L, respectively. In addition to the increased inflammatory indexes in all, we found that triiodothyronine decreased in all cases, and free triiodothyronine decreased in 6 of them. Coronary artery abnormalities (3 dilatation) and abdominal abnormalities (2 gallbladder volume increase and 1 bile duct wall thickening) were detected by ultrasonography.

All patients were diagnosed with classical KD and treated with IVIG and oral acetylsalicylic acid. Two of them had IVIG resistance; one was given the second IVIG treatment and the other recurrent patient was given infliximab treatment. Except for the 2 patients of IVIG-refractory KD, the liver biochemical indexes of the remaining 5 patients improved or even returned to normal within 48 h after conducting IVIG treatment, and all of them returned to normal within 1 wk. After the second IVIG and infliximab were given to 2 patients with IVIG-refractory KD, the liver biochemical indexes returned to normal within 4 d.

Pediatricians should be aware that febrile cholestatic jaundice may be the atypical first symptom of Kawasaki disease, and liver function usually improves or even returns to normal within 1 wk after effective treatment of KD.