Kawasaki disease (KD) has shown a marked increase in trend over the globe, especially within the last two decades. Kawasaki disease is often seen in the paediatric population below five years old, while it is rare for those who are beyond that age. Up to this date, no exact causes has been identified although KD was found more than half a century ago. The underlying pathogenesis of the disease is still unelucidated, and researchers are trying to unlock the mystery of KD. To further complicate the diagnosis and the prompt management, a specific biomarker for the diagnosis of KD is yet to be discovered, making it hard to differentiate between KD and other diseases with a similar presentation. Nonetheless, since its discovery, clinicians and scientists alike had known more about the different clinical aspects of typical KD. Thus, this article intends to revisit and review the various clinical manifestations and laboratory characteristics of KD in order to guide the diagnosis of KD.

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.

IntroductionKawasaki disease (KD) is an acute idiopathic systemic vasculitis with a self- limiting course that predominantly affects children under 5 years old, particularly in the East Asian countries. Nevertheless, to date, the data on KD in Malaysia are limited. This study aimed to evaluate the epidemiology, clinical features, treatment, and outcomes of KD among the pediatric patients admitted to Hospital Canselor Tunku Muhriz (HCTM), Kuala Lumpur, Malaysia.MethodA retrospective cohort study of 66,500 pediatric patients presented at HCTM from the year 2004 to 2021 was conducted.Results62 KD cases out of 66,500 pediatric admissions were reported, with a male-to-female ratio of 1.58 to 1. Majority of KD patients (95.0%) were younger than 5 years old. Prior infection was reported in 5 KD patients (8.1%). Apart from the classical features, manifestations of various organ systems including cardiovascular (16.1%), gastrointestinal (43.5%), neurological (1.61%), musculoskeletal (1.61%), and genitourinary (17.7%) systems were observed. There was a significant association between sterile pyuria and coronary artery aneurysm (CAA) (p < 0.05). Interestingly, abnormal liver parameters (p < 0.05) and incomplete KD (p < 0.05) were significantly related to IVIG resistance.DiscussionThe presence of family history, immunological disorder, and previous infection in our KD patients suggested that there is a possibility of genetic, immunological, and infectious roles in the pathophysiology of KD. IVIG resistance is more likely to occur in KD patients with hepatic dysfunction or incomplete KD presentation. These findings highlighted the significant contribution of laboratory parameters to the prognosis of KD, prompting more in-depth research on the KD scoring systems and their relevance in this country.