Epstein-Barr virus (EBV) causes various acute and chronic diseases. Chronic active EBV infection (CAEBV) is characterized by infectious mononucleosis-like symptoms that persist for more than 6 months with high viral loads in peripheral blood and/or an unusual pattern of anti-EBV antibodies. Severe CAEBV is associated with poor prognosis with severe symptoms, an extremely high EBV-related antibody titer, and hematologic complications that often include hemophagocytic lymphohistiocytosis. However, CAEBV which led to the development of aplastic anemia (AA) has not been reported yet. A 73-year-old woman was admitted to our hospital with intermittent fever, general weakness and elevated liver enzymes. In the serologic test, EBV-related antibody titer was elevated, and real-time quantitative-PCR in peripheral blood showed viral loads exceeding 10(4) copies/microg DNA. Liver biopsy showed characteristic histopathological changes of EBV hepatitis and in situ hybridization with EBV-encoded RNA-1 was positive for EBV. Pancytopenia was detected in peripheral blood, and the bone marrow aspiration biopsy showed hypocellularity with replacement by adipocytes. AA progressed and the patient was treated with prednisolone but deceased 8 months after the diagnosis due to multiple organ failure and opportunistic infection. Herein, we report a rare case of severe CAEBV in an adult patient accompanied by AA and persistent hepatitis.
Aged ; Anemia, Aplastic/*complications ; Carbapenems/therapeutic use ; Chronic Disease ; DNA, Viral/blood ; Epstein-Barr Virus Infections/complications/*diagnosis/pathology ; Female ; Hepatitis/*complications ; Herpesvirus 4, Human/*genetics/isolation & purification ; Humans ; Real-Time Polymerase Chain Reaction ; Severity of Illness Index ; Urinary Tract Infections/drug therapy

Prediction of liver fibrosis progression has a key role in the management of chronic viral hepatitis, as it will be translated into the future risk of cirrhosis and its various complications including hepatocellular carcinoma. Both hepatitis B and C viruses mainly lead to fibrogenesis induced by chronic inflammation and a continuous wound healing response. At the same time direct and indirect profibrogenic responses are also elicited by the viral infection. There are a handful of well-established risk factors for fibrosis progression including older age, male gender, alcohol use, high viral load and co-infection with other viruses. Metabolic syndrome is an evolving risk factor of fibrosis progression. The new notion of regression of advanced fibrosis or even cirrhosis is now strongly supported various clinical studies. Even liver biopsy retains its important role in the assessment of fibrosis progression, various non-invasive assessments have been adopted widely because of their non-invasiveness, which facilitates serial applications in large cohorts of subjects. Transient elastography is one of the most validated tools which has both diagnostic and prognostic role. As there is no single perfect test for liver fibrosis assessment, algorithms combining the most validated noninvasive methods should be considered as initial screening tools.
Age Factors ; Antiviral Agents/therapeutic use ; Biological Markers/blood ; Hepatitis, Chronic/drug therapy/*pathology ; Hepatitis, Viral, Human/drug therapy/*pathology ; Humans ; Liver/ultrasonography ; Liver Cirrhosis/*pathology ; Orthohepadnavirus/genetics ; Risk Factors

OBJECTIVETo explore the pathological and clinical characteristics of children with liver diseases by retrospective study on clinical and liver biopsy pathological data of children with liver diseases.

METHODThis retrospective analysis was performed at Beijing No. 302 Hospital among 3 932 children with liver diseases who visited the hospital from January 2001 to December 2012. The kinds of diseases were compared with the results of 1983-2000.

RESULT(1) Liver biopsy was successful in 99.72% (3 932/3 943) of cases of 2001-2012 group, complications occurred in 31 children only. (2) Of the 3 932 cases, 2 647 (67.32%) had hepatitis , non-hepatotropic viral hepatitis and non viral liver disease were seen in 365 cases (9.28%), and 920 cases (23.4%), respectively. Among 2 647 cases with viral hepatitis, 2 115 were hepatitis B (79.90%), 521 hepatitis C (19.69%), 7 were hepatitis A (0.26%) and 4 hepatitis E (0.15%), respectively. (3) In 2001-2012 group, the degrees of inflammatory activity (>G2) of liver were seen in 9.57% (202/2 111) patients with hepatitis B, while 23.57% (132/560) in 1983-2000 group. There was significant difference between the two groups (χ(2)=80.36, P=0.00 ). (4) Significant difference was observed in the rate of non viral liver disease between 2001-2012 group (23.40%, 920/3 932) and 1983-2000 group (9.61%, 98/1 020) (χ(2)=93.46, P=0.00). In 2001-2012 group, including 46 kinds of diseases, which were significantly higher than those of 1983-2000 group (18 kinds). In 2000-2012, the main causes of diseases were liver degeneration (18.26%, 168/920), drug-induced liver injury (13.59%, 125/920), fatty liver (8.80%, 81/920) and liver glycogen accumulation disease (8.70%, 80/920). While in 1983-2000 group, the main causes were liver degeneration (20.41%, 20/98), fatty liver (16.33%, 16/98), glycogen storage disease (10.20%, 10/98) and myopathy (9.18%, 9/98).

CONCLUSIONLiver biopsy in children is safe and feasible. Hepatitis B virus was ranked first in children with liver diseases in 2001-2012 group. The kinds of non viral hepatic disorders had changed and extended.

Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; Female ; Hepatitis B ; pathology ; Hepatitis, Viral, Human ; pathology ; Hepatolenticular Degeneration ; epidemiology ; pathology ; Humans ; Infant ; Liver ; pathology ; Liver Diseases ; pathology ; Liver Function Tests ; Male ; Retrospective Studies

Recurrence of viral hepatitis after liver transplantation (LT) can progress to graft failure and lead to a decrease in long-term survival. Recently, there have been remarkable improvement in the treatment of chronic hepatitis B (CHB) using potent antiviral agents. Combination of hepatitis B immunoglobulin and potent antiviral therapy has brought marked advances in the management of CHB for liver transplant recipients. Post-transplant antiviral therapy for hepatitis C virus infection is generally reserved for patients showing progressive disease. Acheiving a sustained virological response in patients with LT greatly ameliorates graft and overall survival, however this only occurs in 30% of transplant recipient using pegylated interferon and ribavirin (RBV). Direct acting antivirals such as protease inhibitors, polymerase or other non-structural proteins inhibitors are anticipated to establish the new standard of care for transplant recipients. In liver transplant recipients, hepatitis E virus infection is an uncommon disease. However, it can lead to chronic hepatitis and cirrhosis and may require retransplantation. Recently, 3-month course of RBV monotherapy has been reported as an effective treatment. This review focuses on the recent management and therapeutic approaches of viral hepatitis in liver transplant recipient.
Antiviral Agents/therapeutic use ; Hepatitis B/drug therapy/pathology/surgery ; Hepatitis C/drug therapy/pathology/surgery ; Hepatitis E/drug therapy/pathology/surgery ; Hepatitis, Viral, Human/drug therapy/pathology/*surgery ; Humans ; *Liver Transplantation ; Recurrence

BACKGROUND/AIMS: It is well-known that patients with chronic liver disease commonly have nutritional deficiency. In the present study, we investigated the differences in malnutrition risk between patients with liver cirrhosis and viral hepatitis carrier. METHODS: Patients with chronic liver disease who have been hospitalized at Konyang University Hospital from May 2012 to April 2013 were analyzed retrospectively. The malnutrition risk was divided into three categories (low, intermediate, and high) according to Konyang University Hospital Malnutrition Screening, which include BMI, serum albumin, total lymphocyte count (TLC), hemoglobin, weight change, appetite, and dysphagia, within 24 hours of hospitalization. RESULTS: Among a total of 460 patients, 313 had liver cirrhosis (LC), and 147 were viral hepatitis carriers. Age (p<0.001), serum albumin level (p<0.001), TLC (p=0.011), hemoglobin (p<0.001) and serum cholesterol level (p=0.005) were significant different between the two groups. However, there were no difference in height, weight, and BMI. The malnutrition risk of the patients with viral hepatitis carrier was significantly lower than that of the patients with LC (p<0.001). In addition, among 313 patients with LC, malnutrition risk of the patients with Child-Pugh class A (n=150) was significantly lower than that of the patients with class B (n=90) or C (n=73) (p<0.001). CONCLUSIONS: Viral hepatitis carrier group had significantly lower malnutrition risk than LC group. The risk of malnutrition in Child-Pugh class A group was significantly lower than that in class B or C group.
Adult ; Aged ; Body Mass Index ; Cholesterol/blood ; Female ; Hemoglobins/analysis ; Hepatitis, Viral, Human/*pathology ; Hospitals, University ; Humans ; Liver Cirrhosis/*pathology ; Lymphocyte Count ; Male ; *Malnutrition ; Middle Aged ; Nutritional Status ; Retrospective Studies ; Risk ; Serum Albumin/analysis ; Severity of Illness Index

BACKGROUND/AIMS: The aim of this study was to describe the types and causes of liver disease in patients from a single community hospital in Korea between April 2005 and May 2010. METHODS: A cohort of patients who visited the liver clinic of the hospital during the aforementioned time period were consecutively enrolled (n=6,307). Consistent diagnostic criteria for each liver disease were set by a single, experienced hepatologist, and the diagnosis of all of the enrolled patients was confirmed by retrospective review of their medical records. RESULTS: Among the 6,307 patients, 528 (8.4%) were classified as acute hepatitis, 3,957 (62.7%) as chronic hepatitis, 767 (12.2%) as liver cirrhosis, 509 (8.1%) as primary liver cancer, and 546 (8.7%) as a benign liver mass or other diseases. The etiologies in the acute hepatitis group in decreasing order of prevalence were hepatitis A (44.3%), toxic hepatitis (32.4%), other hepatitis viruses (13.8%), and cryptogenic hepatitis (9.1%). In the chronic hepatitis group, 51.2% of cases were attributed to viral hepatitis, 33.3% to nonalcoholic fatty liver disease, and 13.0% to alcoholic liver disease (ALD). Of the cirrhoses, 73.4% were attributable to viral causes and 18.1% to alcohol. Of the hepatocellular carcinoma cases, 86.6% were attributed to viral hepatitis and 11.6% to ALD. Among the benign tumors, hemangioma comprised 52.2% and cystic liver disease comprised 33.7%. CONCLUSIONS: Knowledge of the current status of the type and cause of liver disease in Korea may be valuable as a basis for evaluating changing trends in liver disease in that country.
Acute Disease ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Alcohol Drinking/adverse effects ; Carcinoma, Hepatocellular/epidemiology/etiology/pathology ; Chronic Disease ; Cohort Studies ; Fatty Liver/epidemiology ; Female ; Hepatitis/epidemiology ; Hepatitis, Viral, Human/complications/epidemiology ; Humans ; Liver Cirrhosis/epidemiology/etiology ; Liver Diseases/*diagnosis/epidemiology ; Liver Diseases, Alcoholic/complications/epidemiology ; Liver Neoplasms/epidemiology/etiology/pathology ; Male ; Middle Aged ; Prevalence ; Republic of Korea/epidemiology ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate clinicopathological features of DiGeorge syndrome (DGS).

METHODThe clinical features, histological and immunohistochemical findings were analyzed in 5 cases of DGS by autopsy.

RESULTSFive cases of DGS in male infants aged 4 days, 1 month, 7 months, 10 months, and 13 months respectively. Gross and microscopic observations revealed that thymic cortex was depleted of lymphocytes or showed few, dispersed lymphocytes. The thymic medulla showed predominantly epithelial cells with calcified Hassall bodies as well as lymphocyte depletion. T lymphocytes were also scarce in the tonsils, lymph nodes, spleen, and mucosa-associated lymphatic tissue of ileum. In addition, 3 of the 5 patients also showed parathyroid aplasia or dysplasia, and congenital hypertrophy of the ventricular septum.

CONCLUSIONSThe pathological changes indicate that clinicians should be aware of defects of immune system if the infants suffer from severe infections. Pathologists should recognize the importance of abnormalities of lymphohematopoietic tissues in the diagnosis of primary immunodeficiency diseases such as DGS.

Autopsy ; DiGeorge Syndrome ; immunology ; pathology ; virology ; Hepatitis, Viral, Human ; pathology ; Humans ; Hypertrophy, Left Ventricular ; pathology ; Infant ; Infant, Newborn ; Lymphocyte Count ; Male ; Parathyroid Glands ; pathology ; Pneumonia, Viral ; pathology ; T-Lymphocytes ; immunology ; pathology ; Thymus Gland ; pathology

Parvovirus B19 infection is known to cause chronic anemia in immunocompromised hosts, including organ transplant recipients. We report the first case of liver transplant recipient with parvovirus B19-induced pure red cell aplasia in Korea. A 57-yr-old female patient with hepatocellular carcinoma due to hepatitis C virus received a liver transplantation. Two months later, anemia developed and she received periodic red blood cell transfusions. However, chronic anemia persisted and bone marrow examination was performed 8 months after transplantation. Bone marrow aspiration smears showed markedly reduced erythroid precursors with atypical giant pronormoblasts and nuclear remnants with viral inclusions, and characteristic lantern cells were observed in biopsy sections. In addition, parvovirus B19 DNA PCR was positive. She was diagnosed as parvovirus B19-induced pure red cell aplasia and her anemia was improved following intravenous immunoglobulin therapy.
Blood Transfusion ; Bone Marrow/pathology ; Carcinoma, Hepatocellular/etiology/therapy ; DNA, Viral/analysis ; Female ; Hepatitis C/complications/diagnosis ; Humans ; Immunocompromised Host ; Immunoglobulins/therapeutic use ; Liver Neoplasms/etiology/therapy ; Liver Transplantation ; Middle Aged ; Parvoviridae Infections/complications/*diagnosis ; *Parvovirus B19, Human/genetics ; Red-Cell Aplasia, Pure/*diagnosis/therapy/virology

Animals ; Arachidonate 5-Lipoxygenase ; metabolism ; Cyclooxygenase 2 ; metabolism ; Fatty Liver ; enzymology ; pathology ; Hepatitis, Viral, Human ; enzymology ; pathology ; Humans ; Liver Diseases ; enzymology ; pathology ; Liver Diseases, Alcoholic ; enzymology ; pathology ; Liver Neoplasms ; prevention & control

Adolescent ; Adult ; Aged ; Antithyroid Agents ; administration & dosage ; adverse effects ; therapeutic use ; Biomarkers ; blood ; Female ; Hepatitis B ; complications ; pathology ; Hepatitis, Viral, Human ; complications ; pathology ; Humans ; Hyperthyroidism ; complications ; drug therapy ; Liver Function Tests ; Male ; Methimazole ; administration & dosage ; adverse effects ; therapeutic use ; Middle Aged ; Propylthiouracil ; administration & dosage ; adverse effects ; therapeutic use ; Retrospective Studies ; Severity of Illness Index ; Thyroid Function Tests ; Young Adult