Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Peripheral blood mononuclear cells (PBMC, n = 26), formalin-fixed paraffin-embedded liver tissues (n = 11) and saliva (n = 15) of primary biliary cirrhosis (PBC) patients were used for the detection of Epstein-Barr virus (EBV) sequences by polymerase chain reaction (PCR) assay. The semiquantitative analysis of EBV-DNA was also carried out in a reconstructive experiment using an EBV-infected cell line. The PBMCs of PBC patients showed increased levels of EBV-DNA (61%) in contrast to chronic active hepatitis patients (19%), liver cirrhosis patients (14%) and healthy individuals (11%). Furthermore, formalin-fixed paraffin-embedded liver tissues, as well as saliva from PBC patients, also demonstrated increased levels of EBV-DNA when compared to healthy individuals and those with other liver diseases. The increased levels of EBV-DNA in the PBMC, liver tissue and saliva of the PBC patients suggest that those patients may have a depressed immune function against EBV infection.
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PMID:Increased expression of Epstein-Barr virus in primary biliary cirrhosis patients. 133 91

We describe a 27-year-old woman who presented with infertility, and over the subsequent 7 years developed chronic Epstein-Barr virus infection, hepatitis A, cirrhosis and nasopharyngeal carcinoma. Serological evidence of chronic infection with Chlamydia, Epstein-Barr virus and hepatitis A virus was documented.
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PMID:Chronic infection with Epstein-Barr virus, Chlamydia and hepatitis A virus, terminating in cirrhosis and nasopharyngeal carcinoma. 254 39

The Epstein-Barr virus is associated with a broad spectrum of lymphoproliferative diseases in liver allograft recipients. To investigate the effects of primary infection in children following liver transplantation and the possible role of Epstein-Barr virus in the pathogenesis of unexplained chronic hepatitis after transplantation, we used an in situ hybridization technique to detect small virus encoded nuclear RNAs in posttransplant specimens. For comparison, other posttransplant complications and diseases occurring in the nontransplanted liver were studied with the same method. We examined 114 specimens in the following categories: (a) children with primary Epstein-Barr virus infection after transplant (n = 25), (b) chronic hepatitis more than 12 mo after transplant (n = 14), (c) rejection (n = 15), (d) normal/near-normal histology more than 12 mo after transplant (n = 13) (e) end-stage acute liver disease (n = 9) and (f) end-stage cirrhosis (n = 38). Thirty-three of 114 specimens had labeling of occasional portal and parenchymal lymphoid cells. These were present in each of the six main diagnostic categories listed above: 2 of 25, pediatric; 6 of 14, chronic hepatitis; 4 of 15, rejection; 1 of 13, normal; 4 of 9, acute; and 16 of 38, cirrhosis. In none of the patients with Epstein-Barr virus-infected cells did lymphoproliferative disease subsequently develop. In conclusion, we have been unable to show an obvious association between Epstein-Barr virus infection and graft dysfunction after liver transplantation. Our results suggest that immunological response to primary infection in children after liver transplantation may be adequate.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epstein-Barr virus in inflammatory diseases of the liver and liver allografts: an in situ hybridization study. 776 22

During the last 31 months, 50 children between 3 months and 15 years of age have undergone living related liver transplantation (LRLT) for end-stage liver diseases (39 biliary atresia, 2 Budd-Chiari syndrome, 2 progressive intrahepatic cholestasis, 3 liver cirrhosis, 1 Wilson disease, 1 protoporphyria, 1 tyrosinemia, and 1 fulminant hepatitis). Combined FK-506 and low-dose steroids were routinely used for immunosuppression. There were seven deaths, two of which were related to infection (Candida pneumonia and Epstein-Barr virus [EBV]-associated lymphoproliferative syndrome [LPS]). Five patients had a bacterial infection, all of which were associated with surgical complications. Three patients had Candida infection, all of which were malnourished, had biliary atresia, and had been managed with prolonged antibiotics against obstinate ascending cholangitis. There were 14 symptomatic viral infections (1 herpes simplex virus, 1 herpes zoster virus, 5 cytomegalovirus [CMV], 6 EBV, and 1 EBV-associated LPS). Three of the five CMV infections appeared in patients whose graft was ABO-incompatible, who were managed with prophylactic OKT-3. Most of the viral infections (except 1 EBV-associated LPS) were minor and were treated successfully. The low incidence and successful treatment of CMV infection are related to the high compatibility and low incidence of allograft rejection in LRLT. Bacterial and fungal infections can be decreased by greater refinement of surgical technique and more aggressive preoperative management. Treatment of EBV infection is still an unsolved problem.
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PMID:Infectious complications in living related liver transplantation. 801 5

An 18-month-old white male infant with X-linked lymphoproliferative disease was evaluated for persistent hepatic dysfunction following primary Epstein-Barr virus infection. A liver biopsy revealed cirrhosis with a dense mononuclear cell infiltrate. These findings were confounding because cirrhosis is not a typical finding in either normal or immunodeficient individuals following infection with Epstein-Barr virus. An alpha 1-antitrypsin level obtained shortly after biopsy was spuriously within the lower limits of the physiologic range. Further investigation demonstrated a homozygous Z phenotype, the classic protease inhibitor variant described in alpha 1-antitrypsin deficiency. A repeat liver biopsy confirmed the presence of a second hereditary disease. This is a unique concurrence of two uncommon genetic disorders.
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PMID:Alpha 1-antitrypsin deficiency in a child with X-linked lymphoproliferative disease. 930 36

We report the case of a 64-year-old man who presented with a hepatic mass and macronodular cirrhosis. The pathologic findings revealed a lymphoepithelioma-like carcinoma arising in the hepatobiliary tract that was morphologically identical to nasopharyngeal undifferentiated carcinoma. However, this tumor was not associated with Epstein-Barr virus infection in molecular studies. Macronodular cirrhosis associated with hepatitis C virus was present in the background liver.
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PMID:Biliary lymphoepithelioma-like carcinoma not associated with Epstein-Barr virus. 1023 6

It has been suggested that hepatitis C virus (HCV) infection could be associated with B-cell clonal expansion. The aim of this study was to analyze the relationship between lymphoproliferative disorders and HCV infection in liver transplant recipients. We studied 157 patients receiving a liver transplant between January 1989 and May 1997 with a follow-up longer than 3 months. The incidence of posttransplant lymphoproliferative disorders (PTLDs) was analyzed with reference to the indication for liver transplantation, the induction and maintenance immunosuppression, the incidence of acute rejection episodes, and Epstein-Barr virus (EBV) infection. Six PTLDs occurred after a median posttransplant follow-up of 7 months (3.8%). Four of the 6 PTLDs occurred among the 38 patients transplanted for HCV-related cirrhosis, and 2 PTLDs occurred in the 119 patients receiving a liver transplant for non-HCV liver diseases (10.5% vs. 1.7%, respectively; P =.03). The 4-year probability of PTLD was significantly higher in patients receiving a liver transplant for HCV-related cirrhosis than non-HCV liver diseases (12.3% vs. 2.2%, respectively; P =.015). Patients receiving a liver transplant for HCV-related cirrhosis were more likely to receive antithymocyte globulins (ATG). However, in patients treated with ATG, the 4-year probability of PTLD was higher among those patients receiving a liver transplant for HCV-related cirrhosis than for non-HCV liver diseases (27.1% vs. 6.4%, respectively; P =.08). EBV gene products were detected in tumor tissues in 3 of 4 patients with HCV-associated PTLD. Our data suggest that, in addition to EBV infection, 2 mutually nonexclusive factors, i.e., the use of ATG and HCV infection, could play a role in the occurrence of PTLD after a liver transplant for HCV-related cirrhosis.
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PMID:Role of hepatitis C virus in lymphoproliferative disorders after liver transplantation. 1072 16

This is the first reported case of lymphoproliferative disease presenting with adrenal insufficiency after liver transplantation. A 38-year-old white man was admitted 8 months after transplantation for cryptogenic cirrhosis with fever (38-39 degrees C), chills, cough, and dyspnea. His blood pressure was 100/70 mm Hg, there was pallor of the conjunctiva, and a lymph node was palpable in the left groin. Laboratory analyses revealed the following values: serum sodium concentration (112 mmol/L), potassium (5.4 mmol/L), hemoglobin (7.8 g/L), white blood cell count (7.7 x 10(9)/L), glucose 3.9 (mmol/L), and mildly elevated liver functions. Abdominal ultrasound showed multiple hypoechoic solid-appearing lesions throughout the liver and spleen. Results of a biopsy specimen of the groin node confirmed polymorphic B-cell lymphoma. A negative Epstein- Barr virus screen before transplant became positive. The patient's fever increased to 40 degrees C. He subsequently developed sepsis and later, multiple organ failure. Autopsy confirmed extensive abdominal disease. The adrenal glands had been completely replaced by the tumor. Primary Epstein-Barr virus infection is associated with posttransplant lymphoproliferative disease. Replacement of the adrenal glands with a tumor produces a clinical picture of adrenal insufficiency.
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PMID:Posttransplant lymphoproliferative disease presenting as adrenal insufficiency: case report. 1598 81

Epstein-Barr virus (EBV) infection can cause diverse renal manifestations ranging from microscopic hematuria to acute renal failure. Membranous nephropathy (MN) is an uncommon and usually secondary cause of nephrotic syndrome in children, and has been reported after chronic infections and antigenemia. We report two pediatric cases of secondary MN associated with acute and chronic systemic EBV infection. Patient 1 had a liver transplant for cirrhosis due to biliary atresia and developed chronic EB viremia. Membranous nephropathy occurred 3 years later and with aggressive therapy has partially subsided, in temporal association with a drop in blood EBV PCR levels. The other patient had a primary immunodeficiency and developed a lymphoproliferative disorder attributed to EBV. Nephrotic syndrome developed at initial presentation and was associated with MN on biopsy. The patient cleared the virus from blood, which was associated with eventual resolution of the MN. We postulate that EB viremia in patients lacking a fully competent immune system, but without a renal allograft, may create a susceptible environment for chronic systemic EB antigenemia that can then lead to immune-complex MN in the kidney. The association of EBV with renal histological changes consistent with MN has been suggested but not directly described before.
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PMID:Systemic Epstein-Barr virus infection associated with membranous nephropathy in children. 1655 Jul 46

Lymphoepitelioma is a particular form of undifferentiated carcinoma, characterized by a prominent lymphoid stroma, originally described in the nasopharynx. Lymphoid stroma-rich carcinomas arising in other organs have been termed lymphoepithelioma-like carcinoma (LELC). In the liver, primary LELCs are very rare, and the majority has been identified as cholangiocarcinomas. Here a rare case of lymphoepithelioma-like hepatocellular carcinoma (HCC) is described. A 47-year old woman presented with abdominal pain. Ultrasonography revealed a liver nodule, 2.2 cm in diameter, localized in the right lobe, adjacent to the gallbladder. Viral markers for hepatic B virus (HBV), hepatic C virus (HCV) and Epstein-Barr virus (EBV) were negative. The nodule was hypoechogenic. The patient underwent surgery, with resection of the nodule. Histology showed hepatocellular carcinoma, characterized by a prominent lymphoid infiltrate. At immunocytochemistry, tumor cells were reactive for Hep Par1 and glypican 3. Immunophenotyping of tumor infiltrating lymphocytes evidenced the predominance of CD8+ cytotoxic suppressor T cells. The postoperative clinical outcome was favorable and the patient was recurrence-free 15 mo after resection. This case, to the best of our knowledge, is the first reported non EBV and non cirrhosis-associated lymphoepithelioma-like hepatocellular carcinoma. The association between the lack of EBV infection, the absence of cirrhosis, a "cytotoxic profile" of the inflammatory infiltrate and a good prognosis could identify a variant of lymphoepithelioma-like HCC with a favorable clinical outcome.
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PMID:Lymphoepitelioma-like hepatocellular carcinoma: a case report and a review of the literature. 1869 86


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