BACKGROUND: Fas is a member of the tumor necrosis factor (TNF)/nerve growth factor (NGF) receptor family. Triggering of the Fas receptor pathway by its ligand results in apoptosis. Soluble Fas consists of the extracellular region of Fas receptor and it binds to Fas ligand to inhibit the Fas and Fas ligand induced apoptosis. Recently some evidence indicates that the Fas/Fas ligand system represents an important pathway responsible for the induction of apoptosis in bone marrow CD34+ cells of patients with aplastic anemia. METHODS: We measured serum soluble Fas levels in 27 patients with aplastic anemia at diagnosis using ELISA to define the status of soluble Fas in this disorder. RESULTS: Levels of serum soluble Fas in patients with aplastic anemia were lower com-pared with that of normal healthy controls. No difference was noted in the serum soluble Fas levels according to severity of disease. No correlation was found between serum soluble Fas levels and hematologic parameters at diagnosis such as neutrophil count, lymphocyte count, platelet count and corrected reticulocyte count. CONCLUSION: These results indicate that serum soluble Fas levels are decreased in patients with aplastic anemia. Further studies recruiting more patients and measuring Fas receptor on peripheral blood lymphocyte subsets and bone marrow CD34+ cells concomitantly may be helpful to determine pathophysiology of bone marrow failure.
Anemia, Aplastic* ; Antigens, CD95 ; Apoptosis ; Bone Marrow ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Fas Ligand Protein ; Humans ; Lymphocyte Count ; Lymphocyte Subsets ; Neutrophils ; Platelet Count ; Reticulocyte Count ; Tumor Necrosis Factor-alpha

Dengue fever/dengue shock syndrome is an acute febrile illness caused by a Flaviviridae virus. Dengue virus infection can cause a wide spectrum of illness, and disease is characterized by sudden onset of high fever, chill, severe headache, skin rash, general malaise and thrombocytopenia with hemoconcentration. Dengue fever is endemic in most tropical areas of world, including the Caribbean, Central and South America, Africa, and Southeast Asia. Tourists to these areas are liable to infection. We experienced a Korean female who returned to Korea from Myanmar in severely ill state. She was confirmed serologically to be inblieted with Dengue shock syndrome. In spite of intensive medical care, she died of Dengue shock syndrome. We emphasize that favorable prognosis depends mainly on the early recognition and treatment of shock.
Africa ; Asia, Southeastern ; Caribbean Region ; Dengue Virus ; Dengue* ; Exanthema ; Female ; Fever ; Flaviviridae ; Headache ; Humans ; Korea ; Myanmar* ; Prognosis ; Severe Dengue* ; Shock ; South America ; Thrombocytopenia

Dengue fever/dengue shock syndrome is an acute febrile illness caused by a Flaviviridae virus. Dengue virus infection can cause a wide spectrum of illness, and disease is characterized by sudden onset of high fever, chill, severe headache, skin rash, general malaise and thrombocytopenia with hemoconcentration. Dengue fever is endemic in most tropical areas of world, including the Caribbean, Central and South America, Africa, and Southeast Asia. Tourists to these areas are liable to infection. We experienced a Korean female who returned to Korea from Myanmar in severely ill state. She was confirmed serologically to be inblieted with Dengue shock syndrome. In spite of intensive medical care, she died of Dengue shock syndrome. We emphasize that favorable prognosis depends mainly on the early recognition and treatment of shock.
Africa ; Asia, Southeastern ; Caribbean Region ; Dengue Virus ; Dengue* ; Exanthema ; Female ; Fever ; Flaviviridae ; Headache ; Humans ; Korea ; Myanmar* ; Prognosis ; Severe Dengue* ; Shock ; South America ; Thrombocytopenia

Endobronchial tuberculosis is defined as a specific inflammation of the trachea or major bronchi caused by the tubercle bacillus. It is recognized as one of the most common and serious complication of pulmonary tuberculosis. A diagnosis of endobronchial tuberculosis is difficult due to the diversity of radiological patterns. But, it is still relatively common disease in korea. Endobronchial tuberculosis as a cause of the acute respiratory distress syndrome (ARDS) is quite rare. The mortality rate of ARDS is still high in korea. The detection and early elimination of the causes for ARDS at the initial stage can result in a more favorable prognosis. So, patients with ARDS, especially due to endobronchial tuberculosis or other form of tuberculosis, should be treated with antituberculous drugs as soon as possible. We experienced a young female with complaints of sudden onset dyspnea, mild fever. In this case the clinical features, laboratory data and radiologic findings allowed an initial presentation of ARDS. The ARDS was defined by the American-Europian Consensus Conference 19921-3). The cause of ARDS was revealed endobronchial tuberculosis. We started antituberculosis medication and steroid injection quickly, which resulted in good prognosis. We emphasize the prognosis depends mainly on the early recognition and treatment of endobronchial tuberculosis.
Bacillus ; Bronchi ; Consensus ; Diagnosis ; Dyspnea ; Female ; Fever ; Humans ; Inflammation ; Korea ; Mortality ; Prognosis ; Respiratory Distress Syndrome, Adult* ; Trachea ; Tuberculosis ; Tuberculosis, Pulmonary

BACKGROUND: Because of widespread and long-term use of lead, many lead poisoning cases were reported. Especially, in Korea including Southeast Asia, we observed non-occupational lead poisoning cases by herbal medication. METHODS: We identified total 45 cases of lead poisoning through Medric search, from 1973 to 2002. Bibliographies of relevant articles were reviewed. We arranged cases by attributes following 1) reported year, 2) age, 3) sex, 4) exposure sources, 5) exposure duration, 6) clinical manifestation, 7) treatments. RESULTS: The average age of the patients was 41.4 +/- 15.7 year-old. The distribution by sex did not have difference each other. The non-occupational lead poisoning cases were mostly caused by Chinese herbal medication. On the cases of the herbal lead poisoning, average exposure duration was 7.3 +/- 3.8 months and the various dosages were taken from 5 mg to 3 g. The major clinical symptoms were abdominal pain, headache, and constipation. The typical laboratory finding was anemia. Major treatment agent was oral penicillamine. CONCLUSION: To differential diagnose of the lead poisoning from many diseases with similar symptoms is very difficult solely on the clinical aspect. Especially, in non-occupational cases it is more difficult. In Korea, non-occupational lead poisoning cases by herbal medication have been discovered more than in western countries. We reviewed about the lead poisoning cases since 1973, so we want to make an aware of scientific and rational making and management of lead in the health supplementary food and herbal medicine.
Abdominal Pain ; Anemia ; Asia, Southeastern ; Asian Continental Ancestry Group ; Constipation ; Headache ; Herbal Medicine ; Humans ; Korea* ; Lead Poisoning* ; Penicillamine

BACKGROUND/AIMS: Epigenetic silencing of DNA repair genes, O6-methylguanine-DNA methyltransferase (MGMT), hMLH1 and hMSH3, by promoter hypermethylation have been observed in various cancers. However, the relationship between hypermethylation of DNA mismatch repair genes and microsatellite instability (MSI) has not been studied in hepatocellular carcinoma (HCC) associated with cirrhosis. METHODS: We investigated the methylation pattern of CpG islands of 3 genes using methylation-specific PCR (MSP) and MSI in 40 patients with paired hepatocellular carcinoma and associated cirrhosis. RESULTS: hMSH3 and MGMT were the most methylated genes in both cirrhosis (70% and 68%, respectively) and HCC (75% and 73%, respectively). The methylation of hMLH1 was rarely found in both cirrhosis (8%) and HCC (5%). Gene promoters methylated in cirrhosis were also methylated in HCC with the exception of 9 cases found to be methylated either in cirrhosis or HCC. Of 40 cases of HCC associated with cirrhosis, three had MSI-positive phenotype in which two were MSI-low and one was MSI-high. One MSI-positive phenotype was present both in cirrhosis and in HCC, while two were only in HCC. There was no significant correlation between aberrant DNA methylation of mismatch repair genes and MSI status in HCC associated with cirrhosis. Immunohistochemical expressions of hMLH1, MGMT, and hMSH3 proteins were present in 16 (40%), 6 (15%), and 11 (28%) of 40 cases of HCC respectively. There was no significant correlaton between the aberrant DNA methylation of mismatch repair genes and clinical characteristics such as histological differentiation, postoperative recurrence and mortality. CONCLUSIONS: The methylation of MGMT and hMSH3 among DNA repair genes are frequent, but those of hMLH1 and MSI is very rare in both cirrhosis and HCC. There is no significant correlation between the methylation of DNA repair genes and clinical characteristics of HCC.
Adaptor Proteins, Signal Transducing/genetics ; Adult ; Aged ; Carcinoma, Hepatocellular/complications/diagnosis/*genetics ; *DNA Methylation ; DNA Modification Methylases/genetics ; *DNA Repair ; DNA Repair Enzymes/*genetics ; DNA-Binding Proteins/genetics ; Female ; Humans ; Liver Cirrhosis/complications/diagnosis/*genetics ; Liver Neoplasms/complications/diagnosis/*genetics ; Male ; *Microsatellite Instability ; Middle Aged ; Nuclear Proteins/genetics ; Tumor Suppressor Proteins/genetics

Mitochondrial myopathies are diseases caused by defects in metabolic pathway of mitochondria. Mitochondrial myopathy is known as one of the causes of recurrent myoglobinuria, while clinically, rarely causes acute renal failure requiring medical treatments. We report a case of rhabdomyolysis and acute renal failure associated with mitochondrial myopathy. A 58-year-old male was presented with dyspnea and hypotensive shock. The patient had a history of recurrent dark colored urine and cramping leg pain after prolonged fasting. Laboratory findings showed hyperkalemia, azotemia, metabolic acidosis, and elevated AST, ALT, and creatinine kinase. He had no history of trauma or medication. Muscle biopsy showed "ragged red fibers" in modified Gomori staining. On electron microscope, increased number of mitochondria and abnormal mitochondria were seen. He received hemodialysis and his renal function recovered after 1 month.
Acidosis ; Acute Kidney Injury* ; Azotemia ; Biopsy ; Creatinine ; Dyspnea ; Fasting ; Humans ; Hyperkalemia ; Leg ; Male ; Metabolic Networks and Pathways ; Middle Aged ; Mitochondria ; Mitochondrial Myopathies* ; Muscle Cramp ; Myoglobinuria ; Phosphotransferases ; Renal Dialysis ; Rhabdomyolysis* ; Shock

BACKGROUND: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. The disease is relatively common in western countries, but so far, only about 20 cases have been reported in Korea. Thus, the purpose of our prospective study was to evaluate the clinical characteristics of PBC in Korea. METHODS: Between October 1994 and February 1999, 16 patients diagnosed as PBC at our department were enrolled in this study. We analyzed these patients for the distribution of age and sex, initial symptoms and signs, associated disorders, laboratory, endoscopic, and radiologic and histologic findings. RESULTS: Of the 16 patients, the ratio of male to female was 1:7 and the average age was 57.5 years. Pruritus (37.5%) was the most frequent presenting symptom followed by xerostomia and xerophthalmia (12.5%), jaundice (6.3%), chronic fatigue (6.3%), melena (6.3%). Associated disorders were Sj gren's syndrome (25%), arthropathy (12.5%), Raynaud's phenomenon (6.3%) and diabetes (6.3%). Abnormalities of liver function were found frequently. Antimitochondrial antibody (100%), antinuclear antibody (31.25%) and rheumatoid factor (31.25%) were found. Long-term administration of UDCA improved both clinical and biochemical signs in most patients, however, 2 patients experienced exacerbation during UDCA therapy. CONCLUSIONS: We experienced 16 cases of PBC within a short period. It is expected that we can find these patients more frequently if we suspect this disease in patients with pruritus and unexplained obstructive pattern of liver function abnormality.
Antibodies, Antinuclear ; Fatigue ; Female ; Hemolytic-Uremic Syndrome ; Humans ; Jaundice ; Korea ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Male ; Melena ; Pancreatitis ; Prospective Studies ; Pruritus ; Rheumatoid Factor ; Xerophthalmia ; Xerostomia