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

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 pre...

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Autores principales: Pratap, Krishan, Gardner, Logan S., Gillis, David, Newman, Martin, Wainwright, Dana, Prentice, Roger
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7590489/
https://www.ncbi.nlm.nih.gov/pubmed/33109118
http://dx.doi.org/10.1186/s12876-020-01461-2
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author Pratap, Krishan
Gardner, Logan S.
Gillis, David
Newman, Martin
Wainwright, Dana
Prentice, Roger
author_facet Pratap, Krishan
Gardner, Logan S.
Gillis, David
Newman, Martin
Wainwright, Dana
Prentice, Roger
author_sort Pratap, Krishan
collection PubMed
description 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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spelling pubmed-75904892020-10-27 Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature Pratap, Krishan Gardner, Logan S. Gillis, David Newman, Martin Wainwright, Dana Prentice, Roger BMC Gastroenterol Case Report 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. BioMed Central 2020-10-27 /pmc/articles/PMC7590489/ /pubmed/33109118 http://dx.doi.org/10.1186/s12876-020-01461-2 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Pratap, Krishan
Gardner, Logan S.
Gillis, David
Newman, Martin
Wainwright, Dana
Prentice, Roger
Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title_full Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title_fullStr Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title_full_unstemmed Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title_short Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature
title_sort hepatic predominant presentation of kawasaki disease in adolescence case report and review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7590489/
https://www.ncbi.nlm.nih.gov/pubmed/33109118
http://dx.doi.org/10.1186/s12876-020-01461-2
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