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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary Epstein-Barr virus (EBV) infection is often asymptomatic before five years of age, whereas it is more likely to be symptomatic in older children, adolescents and young adults. The classic triad of symptoms including fever, pharyngitis and lymphadenopathy is well known, but the spectrum of clinical manifestations associated with EBV infection is large and continues to expand, including increasing atypical presentations. This article presents the case of a five-year-old female in which only a protracted and severe itch, resistant to antihistamines, caught the attention of the girl's parents, thus allowing the diagnosis of EBV. Furthermore, EBV related splenomegaly and mild hepatitis as well as thrombocytopenia had an atypical prolonged course.
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PMID:Atypical Epstein-Barr-Virus infection in a 5-year-old girl. 1918 8

Infectious mononucleosis due to Epstein-Barr virus (EBV) infection sometimes causes acute hepatitis, which is usually self-limiting with mildly elevated transaminases, but rarely with jaundice. Primary EBV infection in children is usually asymptomatic, but in a small number of healthy individuals, typically young adults, EBV infection results in a clinical syndrome of infectious mononucleosis with hepatitis, with typical symptoms of fever, pharyngitis, lymphadenopathy, and hepatosplenomegaly. EBV is rather uncommonly confirmed as an etiologic agent of acute hepatitis in adults. Here, we report two cases: the first case with acute hepatitis secondary to infectious mononucleosis and a second case, with acute hepatitis secondary to infectious mononucleosis concomitantly infected with hepatitis A. Both cases involved young adults presenting with fever, pharyngitis, lymphadenopathy, hepatosplenomegaly, and atypical lymphocytosis confirmed by serologic tests, liver biopsy and electron microscopic study.
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PMID:Infectious mononucleosis hepatitis in young adults: two case reports. 1994 39

Signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) is an adaptor molecule containing a Src homology 2 (SH2) domain. SAP is expressed in T cells and natural killer (NK) cells and binds to the cytoplasmic domains of SLAM family receptors, resulting in the subsequent recruitment of Fyn. The SAP (SH2D1A) gene is located on the X chromosome and is responsible for X-linked lymphoproliferative disease, characterized by higher susceptibility to Epstein-Barr virus infection. The SAP-mediated signal is not only essential for the development of NKT cells, i.e. unconventional CD1d-restricted T cells with invariant Valpha14 T cell receptors, but also for the regulation of the function of NK cells and conventional T cells. The role of SAP-mediated signaling in the induction of autoimmune diseases has been analyzed using animal models such as lupus, hepatitis, and graft-versus-host disease and is considered important in their pathogenesis in humans. In this review we highlight the current findings on SAP-mediated signaling in hematopoietic cells and discuss its importance in autoimmune diseases and immunological disorders.
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PMID:Role of SLAM-associated protein in the pathogenesis of autoimmune diseases and immunological disorders. 2004 47

The first case of Kawasaki syndrome presenting as acute severe hepatitis with coagulopathy, in association with an acute Epstein-Barr virus infection is reported. This case supports the hypothesis that Epstein-Barr virus may play a role in the etiology of Kawasaki syndrome and serves as a reminder that a diagnosis of Kawasaki syndrome should be considered for a persistently febrile child, even in the face of an atypical presentation.
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PMID:Acute severe hepatitis with coagulopathy: An unusual presentation of Kawasaki syndrome in association with Epstein-Barr virus. 2008 33

The liver is a special lymphoid organ with its own defense mechanism and it is prone to chronic viral and autoimmune diseases. All constituent liver cells are involved in immune mechanisms against foreign toxins and infectious agents: hepatocytes, Kupffer cells, endothelial cells, hepatic stellate cells and liver resident lymphocytes such as natural killer, natural killer T and dendritic cells. The first line of defense is held up by the innate immune system where the adaptive immune response is more antigen-specific, but needs a longer time to build up. Even in drug injury of the liver, the innate immune system is involved. There is activation of natural killer and natural killer T cells, and liver cell damage is induced mainly by the Fas and FasL pathways. In viral hepatitis, the mechanism of liver injury is mainly immune-mediated since almost none of the hepatitis viruses have turned out to be cytopathic by themselves. Cytotoxic T lymphocytes with CD8 epitopes are the most important effector cell population. The immune defense may be downregulated by regulatory T cell lymphocytes, PD1/PDL-positive lymphocytes or suppressor dendritic cells. In Epstein-Barr virus infection, liver cell injury does not seem to be due to direct antigen-specific CTLs, but the injury is assumingly induced by a bystander reaction from activated lymphocytes in the sinusoids. Autoimmune hepatitis is a paradigm of immune-mediated liver injury and has a special, but not pathognomonic, histopathology. The main effector lymphocytes belong to the CD8 population, which are antigen-specific; however, the mechanisms seem to be suppressed by regulatory T cells.
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PMID:Pathology of immune-mediated liver injury. 2046 Aug 91

A 30-year-old woman with hepatitis for 5 months was admitted to our hospital. She had been given a diagnosis of liver dysfunction 2 years previously, and the hepatitis in this case was believed to be drug-induced. On admission, the patient was asymptomatic. Serologic tests for hepatitis A, B, and C were negative, and the laboratory results showed a WBC count of 7600/mm3 (lymphocytes, 85%), an AST level of 559 U/L, ALT level of 427 U/L, and EBV-DNA of 2.9x10(6) copies/microg DNA. Histopathological examination of the liver biopsy specimens revealed moderate lymphocyte infiltration in the sinusoids and positive Epstein-Barr-encoded RNA (EBER) -lymphocytes. Therefore, chronic active Epstein-Barr virus infection (CAEBV) was diagnosed. However, 9 months after the diagnosis she died of mycotic sepsis. We presume that the patient may have developed CAEBV at the prior diagnosis of liver dysfunction 2 years previously. Therefore, CAEBV associated with liver dysfunction should be considered during the differential diagnosis of patients showing persistent liver dysfunction.
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PMID:[An adult with chronic active Epstein-Barr virus infection associated with repeated liver dysfunction]. 2069 56

The Epstein-Barr virus (EBV) has an important and multifaceted role in liver pathology. As a member of the herpes virus family, EBV establishes a persistent infection in more than 90% of adults. Besides acute hepatitis during primary infection, many clinical syndromes of interest for the hepatologist are associated with EBV infection. The role of EBV in the evolution of chronic hepatitis from hepatotropic viruses is considered. Chronic EBV-associated hepatitis is suspected in immunocompetent adults with compatible serology, suggestive histology and detection of the viral genome in the liver and/or increase of specific circulating cytotoxic T-lymphocytes. EBV is the main cause of post-transplant lymphoproliferative disorders which occur in up to 30% of cases. EBV-driven lymphoproliferative diseases are also recognized in non-immunocompromised patients and liver is involved in up to a third of the cases. Directly implicated in the pathogenesis of different tumors, EBV has a disputable role in hepatocellular carcinoma carcinogenesis. Further research is required in order to establish or reject the role of EBV in human liver cancer. This paper attempts to discuss the range of EBV-associated chronic liver diseases in immunocompetent patients, from mild, self-limiting mononuclear hepatitis to liver cancer.
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PMID:Epstein-Barr virus: silent companion or causative agent of chronic liver disease? 2080 28

We present a case of life-threatening Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with severe hepatitis that was successfully treated by allogeneic stem cell transplantation from an unrelated donor. A 26-year-old woman was admitted to hospital with a high fever and liver dysfunction. Laboratory tests, including bone marrow aspiration, revealed severe HLH that occurred after EBV infection. High-dose methylprednisolone and etoposide therapy did not control the disease. We could control the HLH, but the EBV viremia continued following the CHOPE (cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide) chemotherapy regimen. Therefore, the patient underwent allogeneic bone marrow transplantation from an HLA-matched, unrelated donor. The patient has remained in good condition without disease recurrence for 2 years after bone marrow transplantation. Although there is no consensus regarding allogeneic stem cell transplantation for EBV-HLH, it is the treatment of choice for aggressive EBV-HLH when the patient is refractory to intensive chemotherapy.
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PMID:Unrelated bone marrow transplantation induced long-term remission in a patient with life-threatening Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. 2162 62

Infectious mononucleosis (IM) is one of the representative, usually benign, acute diseases associated with primary Epstein-Barr virus (EBV) infection. IM is generally self-limiting and is characterized mostly by transient fever, lymphadenopathy and hepatosplenomegaly. However, very rarely primary EBV infection results in severe or fatal conditions such as hemophagocytic lymphohistiocytosis together with fulminant hepatitis designated as severe or fatal IM or EBV-associated hemophagocytic lymphohistiocytosis alone. In addition, chronic EBV-associated diseases include Burkitt's lymphoma, undifferentiated nasopharyngeal carcinoma, Hodgkin lymphoma, T-cell lymphoproliferative disorder (LPD)/lymphoma, natural killer-cell LPD including leukemia or lymphoma, gastric carcinoma, pyothorax-associated lymphoma and senile B-cell LPD as well as chronic active EBV infection and LPD/lymphoma in patients with immunodeficiency. The number of chronic life-threatening diseases linked to the EBV infection is increasingly reported and many of these diseases have a poor prognosis. This review will focus on the historical, pathogenetic, diagnostic, therapeutic and prophylactic issues of EBV-associated life-threatening diseases.
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PMID:Acute or chronic life-threatening diseases associated with Epstein-Barr virus infection. 2210 26

A chronic infectious mononucleosis-like illness caused by Epstein-Barr virus (EBV) is called 'chronic active EBV disease', which is defined as an EBV-associated lymphoproliferative disease. This lymphoproliferative disease is rare and predominantly occurs in Japanese children. Between 1998 and 2010, seven adult-onset cases (aged 20-45 years, median 39 years) were identified, which initially presented with inflammatory diseases, including hepatitis, interstitial pneumonitis, uveitis, nephritis and hypersensitivity to mosquito bites. They showed an EBV viral load in the peripheral blood and evidence of EBV infection of T or natural killer (NK) cells. Five cases (71.4%) developed EBV-positive T/NK-cell lymphoma/leukaemia at a median of 5 years (range 1-7 years) after the diagnosis. Although l-asparaginase-containing chemotherapy was effective for the lymphomas, only allogeneic haematopoietic cell transplantation eradicated EBV-infected cells. This observation indicates that persistent EBV infection of T or NK cells defines a distinct disease entity, which provides an underlying condition for EBV-positive T/NK-cell lymphoma/leukaemia.
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PMID:T/NK cell type chronic active Epstein-Barr virus disease in adults: an underlying condition for Epstein-Barr virus-associated T/NK-cell lymphoma. 2224 63


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