Kawasaki Disease with Hepatobiliary Manifestations

Abstract

Background and Objectives: Kawasaki Disease (KD) incidence has been on the rise globally throughout the years, particularly in the Asia Pacific region. KD can be diagnosed based on several clinical criteria. Due to its systemic inflammatory nature, multi-organ involvement has been observed, making the diagnosis of KD more challenging. Notably, several studies have reported KD patients presenting with hepatobiliary abnormalities. Nonetheless, comprehensive data regarding the hepatobiliary manifestations of KD are limited in Malaysia, justifying a more in-depth study of the disease in this country. Thus, in this article, we aim to discuss KD patients in Malaysia with hepatobiliary manifestations. Materials and Methods: A total of six KD patients with hepatobiliary findings who presented at Hospital Canselor Tuanku Muhriz (HCTM) from 2004 to 2021 were selected and included. Variables including the initial presenting signs and symptoms, clinical progress, laboratory investigations such as liver function test (LFT), and ultrasound findings of hepatobiliary system were reviewed and analyzed. Results: Out of these six KD patients, there were two patients complicated with hepatitis and one patient with gallbladder hydrops. Different clinical features including jaundice (n = 3) and hepatomegaly (n = 4) were also observed. All patients received both aspirin and intravenous immunoglobulin (IVIG) as their first-line treatment and all of them responded well to IVIG. The majority of them (n = 5) had a complete recovery and did not have any cardiovascular and hepatobiliary sequelae. Conclusions: Despite KD mostly being diagnosed with the classical clinical criteria, patients with atypical presentations should always alert physicians of KD as one of the possible differential diagnoses. This study discovered that hepatobiliary manifestations in KD patients were not uncommon. More awareness on the epidemiology, diagnosis, and management of KD patients with hepatobiliary manifestations are required to allow for the initiation of prompt treatment, thus preventing further complications.

Jaundice-predominant manifestation of Kawasaki disease in children

“…Although gastrointestinal symptoms are not included in the classical diagnostic criteria, hydrops of the gallbladder and acute febrile jaundice have been reported as atypical findings in some studies ( 10 – 12 ). Suhaini et al suggested that it is crucial to focus on hepatobiliary manifestations in KD ( 13 ), because KD is the second most common cause of acute febrile jaundice and has been reported in up to 20% of cases ( 2 , 14 , 15 ). Our findings highlight the importance of the jaundice-predominant manifestation of KD in infants and children.…”

“…Conversely, Taddio et al found that some KD patients had jaundice but no hydrops of the gallbladder at presentation ( 14 ), whereas Sun et al found that some KD patients with obstructive jaundice who progressed to severe hydrops of the gallbladder had a similar presentation with acute abdomen ( 36 ). It is therefore crucial to detect jaundice clinically ( 13 ). Taken together, our findings indicate that the timely suspicion of KD through the detection of jaundice can prevent further coronary artery complications and acute abdomen among young infants.…”