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Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever
BACKGROUND: Familial Mediterranean Fever is a monogenic autoinflammatory disease, secondary to mutation of MEFV gene, and typically expressed with recurrent attacks of fever, serositis, rash, aphthous changes in lips and/or oral mucosa. Kawasaki Disease, an acute systemic vasculitis with persistent...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6798734/ https://www.ncbi.nlm.nih.gov/pubmed/31627741 http://dx.doi.org/10.1186/s13052-019-0717-8 |
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author | Maggio, Maria Cristina Fabiano, Carmelo Corsello, Giovanni |
author_facet | Maggio, Maria Cristina Fabiano, Carmelo Corsello, Giovanni |
author_sort | Maggio, Maria Cristina |
collection | PubMed |
description | BACKGROUND: Familial Mediterranean Fever is a monogenic autoinflammatory disease, secondary to mutation of MEFV gene, and typically expressed with recurrent attacks of fever, serositis, rash, aphthous changes in lips and/or oral mucosa. Kawasaki Disease, an acute systemic vasculitis with persistent fever (5 or more days), rash, stomatitis, conjunctivitis, lymphadenopathy, changes in extremities, is currently considered a multifactorial autoinflammatory disease. An infection, as Epstein Barr virus, can be the trigger of Kawasaki Disease. CASE PRESENTATION: We describe the clinical case of a 3-year-old boy with Kawasaki disease. Successfully treated with intravenous immune globulin, acetyl salicylate acid, he late developed anaemia and thrombocytopenia. The Epstein-Barr virus infection has been demonstrated and he showed a resolution of the clinical manifestations of Kawasaki disease with the persistence of coronaritis, without aneurisms. However, for the personal and familial history of monthly recurrent attacks of fever, pharyngitis, abdominal pain, the genetic study of MEFV was performed and demonstrated 3 heterozygous mutations of MEFV (E148Q, P369S, R408Q). CONCLUSIONS: Mutations of MEFV can contribute to increase inflammatory expression in other diseases, as Kawasaki disease. |
format | Online Article Text |
id | pubmed-6798734 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-67987342019-10-21 Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever Maggio, Maria Cristina Fabiano, Carmelo Corsello, Giovanni Ital J Pediatr Case Report BACKGROUND: Familial Mediterranean Fever is a monogenic autoinflammatory disease, secondary to mutation of MEFV gene, and typically expressed with recurrent attacks of fever, serositis, rash, aphthous changes in lips and/or oral mucosa. Kawasaki Disease, an acute systemic vasculitis with persistent fever (5 or more days), rash, stomatitis, conjunctivitis, lymphadenopathy, changes in extremities, is currently considered a multifactorial autoinflammatory disease. An infection, as Epstein Barr virus, can be the trigger of Kawasaki Disease. CASE PRESENTATION: We describe the clinical case of a 3-year-old boy with Kawasaki disease. Successfully treated with intravenous immune globulin, acetyl salicylate acid, he late developed anaemia and thrombocytopenia. The Epstein-Barr virus infection has been demonstrated and he showed a resolution of the clinical manifestations of Kawasaki disease with the persistence of coronaritis, without aneurisms. However, for the personal and familial history of monthly recurrent attacks of fever, pharyngitis, abdominal pain, the genetic study of MEFV was performed and demonstrated 3 heterozygous mutations of MEFV (E148Q, P369S, R408Q). CONCLUSIONS: Mutations of MEFV can contribute to increase inflammatory expression in other diseases, as Kawasaki disease. BioMed Central 2019-10-18 /pmc/articles/PMC6798734/ /pubmed/31627741 http://dx.doi.org/10.1186/s13052-019-0717-8 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. 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. |
spellingShingle | Case Report Maggio, Maria Cristina Fabiano, Carmelo Corsello, Giovanni Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title | Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title_full | Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title_fullStr | Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title_full_unstemmed | Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title_short | Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever |
title_sort | kawasaki disease triggered by ebv virus in a child with familial mediterranean fever |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6798734/ https://www.ncbi.nlm.nih.gov/pubmed/31627741 http://dx.doi.org/10.1186/s13052-019-0717-8 |
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