Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Maggio, Maria Cristina, Fabiano, Carmelo, Corsello, Giovanni
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
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
_version_ 1783460124651683840
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
work_keys_str_mv AT maggiomariacristina kawasakidiseasetriggeredbyebvvirusinachildwithfamilialmediterraneanfever
AT fabianocarmelo kawasakidiseasetriggeredbyebvvirusinachildwithfamilialmediterraneanfever
AT corsellogiovanni kawasakidiseasetriggeredbyebvvirusinachildwithfamilialmediterraneanfever