Epstein Barr virus associated hemophagocytic syndrome--a case report.

Virus associated hemophagocytic syndrome (VAHS) are a heterogeneous group of disorders in which viral infection is associated with a proliferation of hemophagocytic histiocytes through the reticuloendothelial system. We report the case of a 21-year-old Korean man who presented to us with high fever, marked hepatosplenomegaly, severe hepatic dysfunction, coagulopathy, pancytopenia and marked panhypogammaglobulinemia. Bone marrow aspiration and biopsy showed histiocytes proliferation with active phagocytosis of red cells and neutrophils. Primary Epstein-Barr (EB) viral infection at presentation was confirmed by the presence of IgM antibody to viral capsid antigen (VCA) with absence of antibody to EB viral nuclear antigen (EBNA). A liver biopsy performed one month after the presentation showed erythrophagocytic histiocytes within the sinusoids. EB virus was demonstrated in the liver biopsy tissue by DNA PCR method, and EBER mRNA in situ hybridization.     

Secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis : report of two cases

Amenorrhea is rarely presented as a manifestation of endocrinological disturbances in patients of chronic hydrocephalus. We describe two cases of secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis. Two female patients of age 30 and 20 yr presented with amenorrhea and increasing headache. Magnetic resonance images revealed marked, noncommunicating hydrocephalus without any tumorous lesion. In one patient, emergent extraventricular drainage was necessary because of progressive neurological deterioration. Each patient underwent surgical intervention for the hydrocephalus-ventriculoperitoneal shunt and endoscopic third ventriculostomy. Both resumed normal menstruation continuing so far with further normal menstrual bleeding. These two cases and others reported in the literature indicated that the surgical intervention for hydrocephalus resolves amenorrhea in all the cases of amenorrhea due to hydrocephalus. The suspected role of the surgery is the correction of increased intracranial pressure, which is an important pathogenetic factor in the development of amenorrhea.     

A case of minute intraductal papillary mucinous tumor of the pancreas presenting with recurrent acute pancreatitis

Intraductal papillary mucinous tumor (IPMT) of the pancreas, a lesion consisting of mucin-producing cells with neoplastic potential, is characterized by duct ectasia, mucin hypersecretion, often extensive papillary intraductal growth, varying degrees of cytologic atypia, and relatively indolent growth. The clinical presentation of IPMT of the pancreas is characterized by chronic or recurrent attacks of abdominal discomfort often in association with low level pancreatic enzyme elevations. Less commonly these lesions may be detected as asymptomatic radiographic abnormalities. Interestingly, a case of a minute IPMT (2 mm in height and 7 mm in length, adenoma) in the main pancreatic duct presenting with acute pancreatitis in a 55 year-old man has been reported in the Japanese literature. Recently, we also experienced a case of a minute IPMT in a branch pancreatic duct causing repeated bouts of acute pancreatitis in a 75 year-old man. A filling defect at the neck of the main pancreatic duct seen on an endoscopic retrograde pancreatogram performed after recovery of the second attack of acute pancreatitis led the patient to undergo an exploratory laparotomy. After a near-total pancreatectomy was carried out, a minute (3 x 7 mm) IPMT of borderline malignancy was discovered in a branch duct at the head portion near the pancreatic neck without any lesions in the main pancreatic duct. Surprisingly, despite the resective surgery the patient died of carcinomatosis 8.5 months after the operation. We herein report a case of a minute but aggressive IPMT of the pancreas with a review of the literature.     

Protective effect of Hypericum perforatum Linn (St. John's wort) against hydrogen peroxide-induced apoptosis on human neuroblastoma cells

The medicinal plant Hypericum perforatum Linn, commonly known as St. John's wort, has been used as an antidepressant. To investigate whether St. John's wort possesses a protective effect against hydrogen peroxide (H(2)O(2))-induced cytotoxicity in neuronal cells, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay, 4,6-diamidino-2-phenylindole staining, terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling assay, flow cytometry analysis, DNA fragmentation assay, and caspase-3 enzyme assay were performed on SK-N-MC human neuroblastoma cells. Cells treated with H(2)O(2) exhibited several apoptotic features, while those pre-treated with St. John's wort prior to H(2)O(2) exposure showed a decreased occurrence of apoptotic features. In addition, pre-treatment with St. John's wort inhibited H(2)O(2)-induced increase in caspase-3 enzyme activity. These results suggest that St. John's wort may exert a protective effect against H(2)O(2)-induced apoptosis in human neuroblastoma cells.     

Spontaneous acute tumor lysis syndrome with advanced gastric cancer

Acute tumor lysis syndrome (TLS) occurs frequently in hematologic malignancies such as high-grade lymphomas and acute leukemia, which are rapidly proliferating and chemosensitive tumors. It occurs rarely in solid tumors and has never been reported in gastric adenocarcinoma. Typical biochemical findings of acute tumor lysis syndrome are hyperuricemia, hyperkalemia, hyperphosphatemia and hypocalcemia in patients with a malignancy. Rapid changes of these electrolytes may cause cardiac arrhythmia, seizure, acute renal failure and sudden death. Therefore, as soon as it is detected, it should be taken care of immediately. Until now almost all cases of TLS associated with solid tumor have developed after cytoreductive therapy in chemosensitive tumors. We report here a case of spontaneous acute tumor lysis in a patient of advanced gastric cancer with hepatic metastases and multiple lymphadenopathy. The biochemical finding of TLS improved with the management and tumor burden also showed slight response to the one cycled combination chemotherapy but the patient died of progressive pneumonia.     

Helicobacter pylori infection in Korea

Helicobacter pylori is a gram-negative bacterium that was first isolated in 1982. Since then, H. pylori infection in humans has been shown to be associated with gastritis, peptic ulcer disease, gastric carcinoma, and mucosa-associated lymphoid tissue (MALT) lymphoma as well. The epidemiology, transmission, and pathogenicity of H. pylori has been a subject of intensive study. Successful treatment improves the cure rate of peptic ulcerations and treatment with antimicrobials also decreases the recurrence rate of these diseases. Better regimens having less toxicity and a good eradication rate have also been developed. A better understanding of the pathophysiologic mechanisms relating to H. pylori induced mucosal damages would result in more options for the prevention of peptic ulcers and carcinogenesis. Korea has a relatively high incidence of H. pylori infection and gastric cancer. Growing interest has developed in view of its importance in being associated with various gastroduodenal diseases. Furthermore, along with a high incidence of H. pylori-related disease in Korea, because the interaction between H. pylori, host factors and environmental factors is important in disease pathogenesis, we need to have precise data on the characteristics of H. pylori-related diseases that occur in Korea. In the present report we review the epidemiology, transmission route, diagnosis, pathogenesis, treatment methods and relationship with gastroduodenal diseases with in special references to basic and clinical data that have been published.     

DTNBP1 haplotype influences baseline assessment scores of schizophrenic in-patients

Dysbindin gene (DTNBP1) has been associated with schizophrenia, but literature findings are inconsistent, and further analyses are required. This study is aimed to investigate if a set of DTNBP1 variations might influence clinic psychotic phenotype or treatment response in a sample of 240 Korean schizophrenic in-patients. Four variants have been selected (rs3213207; rs1011313; rs16876759; rs2619522) on the basis of previous findings of association with schizophrenia, bipolar disorder and antidepressant response. Single marker analysis gave marginal results. Haplotype analysis identified a significant association between A–A (rs3213207(A/G), rs1011313(A/G)) haplotype and lower PANSS total and positive scores at baseline (p = 0.01; 0.02) and at discharge (p = 0.008; 0.005). Covariate analysis revealed a more stable significant association between A–A haplotype and baseline scores. These results suggest a protective effect of A–A haplotype on psychotic positive symptoms at baseline.