Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
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CASE REPORT
Open Access
Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature Krishan Pratap1,2, Logan S. Gardner1,2* , David Gillis1, Martin Newman3, Dana Wainwright1 and Roger Prentice1
Abstract 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. Keywords: Kawasaki, Hepatitis, Liver, Adolescent, Case report
Background Kawasaki Disease (KD) is the most common paediatric vasculitis affecting small to medium arteries [1, 2]. Previously referred to as mucocutaneous lymph node syndrome, the majority of morbidity and mortality in KD stems from cardiac involvement with the development of coronary artery aneurysms and arrhythmias [2].
* Correspondence: [email protected] 1 Department of Clinical Immunology and Allergy, Royal Brisbane and Women’s Hospital, Butterfield Street, Herston, Brisbane, QLD 4029, Australia 2 School of Medicine, University of Queensland, Brisbane, Australia Full list of author information is available at the end of the article
The presentation is typically heralded by a prodromal phase with non-specific viral-like respiratory and gastrointestinal symptoms [1, 2]. Classic findings that form the diagnosis of complete KD as defined by Tomisaku Kawasaki in 1967 are listed in Table 1 [3, 4]. The disease affects between 12 to 215 per 100,000 children under 5 years of age [5–7]. The median age of the disease at diagnosis is 3.4 years and these patients are most likely to present with cl
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