J Korean Pediatr Soc.
2001 Jun;44(6):687-693.
A Case of Chronic Active Epstein-Barr Virus Infection with Coronary Aneurysm
- Affiliations
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- 1Department of Pediatrics, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea.
- 2Department of Diagnostic Pathology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea.
Abstract
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Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific
immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy,
splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis, and uveitis. Cardiovascular
complications are rare in EBV infection. Patients with CAEBV show characteristically high titers of
anti-viral capsid antigen(VCA) IgG antibody and anti-early antigen(EA) antibody, as well as relatively
low titer of anti-EB nuclear antigen(EBNA) antibody. We experienced a case of CAEBV with giant
coronary aneurysms, who was a 6-year-old boy. He had 5 episodes of high fever and cervical
lymphadenopathy and hepatosplenomegaly. The 6 mm sized bilateral coronary aneurysms were detected by
echocardiography at second admission. IgG antibodies to EBV was positive, whereas negative for IgM
antibody. Antibodies to EA and EBNA were also positive. The EBV was detected in lymph node tissue
by in situ hybridization, and in the peripheral blood and bone marrow by the PCR. Treatment has been
done with ganciclovir and interferon (IFN)-alpa for 5 weeks. The EBV-PCR of peripheral blood was
converted to negative from 12th day of treatment. At present he has followed well for two years without
fever and enlargements of lymphnode, except large coronary aneurysm.