Cholestatic hepatitis in immunoglobulin-resistant Kawasaki disease

Gomis, Rosmari Vázquez; Fos, Ignacio Izquierdo; Yañez, Andrea López; Durán, Mabel Mendoza; Robles, María Isabel; Gomis, Consuelo Vázquez; Rosado, José

doi:10.1016/j.gastre.2016.03.002

Cited by 2 publications

(4 citation statements)

References 12 publications

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“…This has been theorised to be due to compressive lymphadenopathy of the porta hepatis [ 16 ]. Less commonly, patients with KD have been found to present with clinical jaundice but without sonographic evidence of hydrops or mechanical obstruction [ 17 – 21 ].…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge there have been five previous reported cases of obstructive jaundice without evidence of hydrops of the gallbladder which we have summarised in Table 3 [ 17 – 21 ]. In these cases, patients demonstrated normal hepatobiliary anatomy on standard imaging modalities with three reports of nonspecific gallbladder/biliary dilatation [ 17 – 21 ]. In all cases LFTs returned to normal ranges following administration of IVIG although long term maintenance of normal liver function was not recorded [ 17 – 21 ].…”

Section: Discussionmentioning

confidence: 99%

“…In these cases, patients demonstrated normal hepatobiliary anatomy on standard imaging modalities with three reports of nonspecific gallbladder/biliary dilatation [ 17 – 21 ]. In all cases LFTs returned to normal ranges following administration of IVIG although long term maintenance of normal liver function was not recorded [ 17 – 21 ]. In three cases, a liver biopsy had been performed which demonstrated evidence of intrahepatic cholangitis and bile duct epithelium necrosis without lobular inflammatory infiltrate, or liver cell necrosis that would be found in a viral aetiology.…”

Section: Discussionmentioning

confidence: 99%

“…Not Refractory. Vázquez et al [ 21 ] 6 + + NR NR NR 3.21 4.02 98.3 – IVIG 2 g/kg/day. Refractory Disease requiring further IVIG 72 h after the 1st dose and 48 h of Methylprednisolone 15-30 mg/kg for 2 days.…”

Section: Discussionmentioning

confidence: 99%

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Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

et al. 2020

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Background Kawasaki Disease (KD) is the most common paediatric vasculitis affecting small to medium arteries. Although the average age of diagnosis is 3.4 years with a well-defined clinical presentation, older patients with KD including adolescent and adult patients demonstrate a less classical presentation with prominent findings including hepatitis, cervical lymphadenopathy, and arthralgia. We describe a case of an adolescent presentation of Kawasaki Disease presenting with a predominantly cholestatic hepatic picture. Case presentation We describe a case of KD in a 16-year-old Caucasian female with predominately hepatic disease that showed resistance to intravenous immunoglobulin (IVIG). The formal diagnosis of KD was made on her 8th day of symptoms. She displayed classical symptoms commencing with fever, followed by peripheral desquamation, strawberry tongue, cervical lymphadenopathy. She became clinically jaundiced with evidence of hepatic artery narrowing on ultrasound that resolved with treatment. Her disease was biphasic and required further IVIG for non-hepatic symptoms. She did not develop coronary aneurysms. Conclusion Significant hepatic dysfunction with clinical jaundice is rare in KD without associated gall bladder hydrops and tends to occur in older patients. We describe such a case and review the five described cases in the literature. Diagnostic delay is more common in adolescent patients and given that the prognosis of KD is closely correlated to diagnostic timing and provision of care, it is important to consider Kawasaki Disease in older demographics especially with undiagnosed hepatic disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

et al. 2020

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Kawasaki Disease with Hepatobiliary Manifestations

et al. 2022

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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.

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Cholestatic hepatitis in immunoglobulin-resistant Kawasaki disease

Cited by 2 publications

References 12 publications

Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

Kawasaki Disease with Hepatobiliary Manifestations

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