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Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever.
Ital J Pediatr 2019; 45(1):129IJ

Abstract

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.

Authors+Show Affiliations

Department of Health Promotion Sciences Maternal and Infantile Care, Internal Medicine and Medical Specialities "G. D'Alessandro", University of Palermo, Palermo, Italy. mariacristina.maggio@unipa.it.U.O. Laboratorio di Genetica Molecolare, Ematologia e Malattie rare del sangue e degli organi emopoietici, Azienda Ospedaliera Ospedali Riuniti "Villa Sofia-Cervello" - Presidio Cervello, Palermo, Italy.Department of Health Promotion Sciences Maternal and Infantile Care, Internal Medicine and Medical Specialities "G. D'Alessandro", University of Palermo, Palermo, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31627741

Citation

Maggio, Maria Cristina, et al. "Kawasaki Disease Triggered By EBV Virus in a Child With Familial Mediterranean Fever." Italian Journal of Pediatrics, vol. 45, no. 1, 2019, p. 129.
Maggio MC, Fabiano C, Corsello G. Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever. Ital J Pediatr. 2019;45(1):129.
Maggio, M. C., Fabiano, C., & Corsello, G. (2019). Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever. Italian Journal of Pediatrics, 45(1), p. 129. doi:10.1186/s13052-019-0717-8.
Maggio MC, Fabiano C, Corsello G. Kawasaki Disease Triggered By EBV Virus in a Child With Familial Mediterranean Fever. Ital J Pediatr. 2019 Oct 18;45(1):129. PubMed PMID: 31627741.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Kawasaki disease triggered by EBV virus in a child with Familial Mediterranean Fever. AU - Maggio,Maria Cristina, AU - Fabiano,Carmelo, AU - Corsello,Giovanni, Y1 - 2019/10/18/ PY - 2019/07/05/received PY - 2019/09/16/accepted PY - 2019/10/20/entrez PY - 2019/10/20/pubmed PY - 2019/10/20/medline KW - Epstein Barr virus KW - Familial Mediterranean fever KW - Kawasaki disease SP - 129 EP - 129 JF - Italian journal of pediatrics JO - Ital J Pediatr VL - 45 IS - 1 N2 - 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. SN - 1824-7288 UR - https://www.unboundmedicine.com/medline/citation/31627741/Kawasaki_disease_triggered_by_EBV_virus_in_a_child_with_Familial_Mediterranean_Fever L2 - https://ijponline.biomedcentral.com/articles/10.1186/s13052-019-0717-8 DB - PRIME DP - Unbound Medicine ER -