Kawasaki disease with a concomitant primary Epstein - Barr virus infection

Abstract Background Kwasaki disease (KD) is the leading cause of acquired heart disease in children in most developed countries. The cause of KD remains unknown. The presumed theory is that KD occurs due to one or more infectious agents who evoke an abnormal immunological response in susceptible ind...

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Main Authors: Nataly Rosenfeld, Diana Tasher, Adi Ovadia, Shirly Abiri, Ilan Dalal
Format: Article
Language:English
Published: BMC 2020-08-01
Series:Pediatric Rheumatology Online Journal
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12969-020-00459-0
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author Nataly Rosenfeld
Diana Tasher
Adi Ovadia
Shirly Abiri
Ilan Dalal
author_facet Nataly Rosenfeld
Diana Tasher
Adi Ovadia
Shirly Abiri
Ilan Dalal
author_sort Nataly Rosenfeld
collection DOAJ
description Abstract Background Kwasaki disease (KD) is the leading cause of acquired heart disease in children in most developed countries. The cause of KD remains unknown. The presumed theory is that KD occurs due to one or more infectious agents who evoke an abnormal immunological response in susceptible individuals. Epstein - Barr virus (EBV) infection has been considered as a suspected causative agent because of the potential effect on the immune system. Case presentation A previously healthy 19 month old boy presented with a 6 day history of fever accompanied by a diffuse macular erythematous rash that appeared 1 day after. The physical examination on admission revealed bilateral non-suppurative conjunctivitis, dry fissured and injected lips without “strawberry” tongue, diffuse macular rash on the trunk, face and limbs, swelling of the hands and feet, and right cervical lymphadenopathy (2 cm in diameter). Following fulfillment of all the clinical criteria, the diagnosis of KD was made and treatment with IVIG 2 g/Kg was administered along with oral aspirin (80 mg/ kg/day). However, despite the treatment, he remained febrile for an additional 2 days with persistent clinical manifestations. Therefore, he received a second 2 g/kg IVIG course with a favorable response. On the 14th day of illness the patient became febrile again and was readmitted. Blood examinations revealed remarkable leukocytosis up to 35.7 X 109/L with 87.3% lymphocytes and the blood smear revealed atypical lymphocytes and monocytes. The liver enzymes were elevated. The serology for infectious mononucleosis from his first admission revealed: IgM CMV (+), IgG CMV (−); IgM VCA EBV (+) IgG VCA EBV (−), IgG EBNA (−). To confirm infectious mononucleosis following the administration of 2 doses of IVIG, serum EBV PCR was performed and was positive (1.6X 103 cp/ml). Conclusions We describe here a case of KD with a concomitant primary EBV infection. To the best of our knowledge, this is the first case in western country that describes KD with acute EBV infection as confirmed by PCR. The case we described stands as a contribution in favor of the possible role of EBV in the development of KD.
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spelling doaj.art-4987585091274ac9ac3e542bcfe3b57b2022-12-22T00:07:51ZengBMCPediatric Rheumatology Online Journal1546-00962020-08-011811310.1186/s12969-020-00459-0Kawasaki disease with a concomitant primary Epstein - Barr virus infectionNataly Rosenfeld0Diana Tasher1Adi Ovadia2Shirly Abiri3Ilan Dalal4Department of Paediatrics, Edith Wolfson Medical CenterDepartment of Paediatrics, Edith Wolfson Medical CenterDepartment of Paediatrics, Edith Wolfson Medical CenterDepartment of Paediatrics, Edith Wolfson Medical CenterDepartment of Paediatrics, Edith Wolfson Medical CenterAbstract Background Kwasaki disease (KD) is the leading cause of acquired heart disease in children in most developed countries. The cause of KD remains unknown. The presumed theory is that KD occurs due to one or more infectious agents who evoke an abnormal immunological response in susceptible individuals. Epstein - Barr virus (EBV) infection has been considered as a suspected causative agent because of the potential effect on the immune system. Case presentation A previously healthy 19 month old boy presented with a 6 day history of fever accompanied by a diffuse macular erythematous rash that appeared 1 day after. The physical examination on admission revealed bilateral non-suppurative conjunctivitis, dry fissured and injected lips without “strawberry” tongue, diffuse macular rash on the trunk, face and limbs, swelling of the hands and feet, and right cervical lymphadenopathy (2 cm in diameter). Following fulfillment of all the clinical criteria, the diagnosis of KD was made and treatment with IVIG 2 g/Kg was administered along with oral aspirin (80 mg/ kg/day). However, despite the treatment, he remained febrile for an additional 2 days with persistent clinical manifestations. Therefore, he received a second 2 g/kg IVIG course with a favorable response. On the 14th day of illness the patient became febrile again and was readmitted. Blood examinations revealed remarkable leukocytosis up to 35.7 X 109/L with 87.3% lymphocytes and the blood smear revealed atypical lymphocytes and monocytes. The liver enzymes were elevated. The serology for infectious mononucleosis from his first admission revealed: IgM CMV (+), IgG CMV (−); IgM VCA EBV (+) IgG VCA EBV (−), IgG EBNA (−). To confirm infectious mononucleosis following the administration of 2 doses of IVIG, serum EBV PCR was performed and was positive (1.6X 103 cp/ml). Conclusions We describe here a case of KD with a concomitant primary EBV infection. To the best of our knowledge, this is the first case in western country that describes KD with acute EBV infection as confirmed by PCR. The case we described stands as a contribution in favor of the possible role of EBV in the development of KD.http://link.springer.com/article/10.1186/s12969-020-00459-0Kawasaki diseaseEpstein- Barr virusIntravenous immunoglobulins
spellingShingle Nataly Rosenfeld
Diana Tasher
Adi Ovadia
Shirly Abiri
Ilan Dalal
Kawasaki disease with a concomitant primary Epstein - Barr virus infection
Pediatric Rheumatology Online Journal
Kawasaki disease
Epstein- Barr virus
Intravenous immunoglobulins
title Kawasaki disease with a concomitant primary Epstein - Barr virus infection
title_full Kawasaki disease with a concomitant primary Epstein - Barr virus infection
title_fullStr Kawasaki disease with a concomitant primary Epstein - Barr virus infection
title_full_unstemmed Kawasaki disease with a concomitant primary Epstein - Barr virus infection
title_short Kawasaki disease with a concomitant primary Epstein - Barr virus infection
title_sort kawasaki disease with a concomitant primary epstein barr virus infection
topic Kawasaki disease
Epstein- Barr virus
Intravenous immunoglobulins
url http://link.springer.com/article/10.1186/s12969-020-00459-0
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