Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder characterized by prolonged bleeding time, ad deficient or absent clot retraction in the presence of normal platelet count. The major underlying abnormality in this disease is grossly defective first-phase aggregation of platelet, which are unresponsive to ADP or other platelet agonists such as epinephrine, collagen, thrombin in any concentration. This disability is caused by a decrease or absence of the platelet membrans glycoprotein IIb-IIIa complex, a member of the integrin family of adhesive receptors involved in cell-cell and cell-matrix fibronectin, and vitronectin On the development of surface labeling technique, a variety of biochemical techniques such as radioimmunoassay, crossed immunoelectrophoresis and SDS-PAGE have been used to study the structure and the function of platelet membrane glycoproteins, and to detect the platelet functional defect. But all of these techniques demand a relatively large amount of homogeneous paletelet population that requires manipulation through isolation and washing procedures before analysis. In order to eliminaste such an intricate procedure, we have applied method for analyzing platelet surface components in whole blood using monoclonal antibody and flow cytometry to recognize the absence of severe reduction of platelet membrane glycoprotien llb-llla complex. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as well as for carriers of this disease. Fow cytometry technique provides a sensitive tool for investigating platelet functional defects caused by altered expression or deficiency of platelet surface proteins.
Adenosine Diphosphate ; Adhesives ; Bleeding Time ; Blood Platelets* ; Clot Retraction ; Collagen ; Electrophoresis, Polyacrylamide Gel ; Epinephrine ; Fibronectins ; Flow Cytometry* ; Glycoproteins ; Hemorrhagic Disorders ; Humans ; Immunoelectrophoresis, Two-Dimensional ; Membrane Glycoproteins* ; Membrane Proteins ; Membranes* ; Platelet Count ; Platelet Membrane Glycoproteins ; Radioimmunoassay ; Thrombasthenia* ; Thrombin ; Vitronectin

Insulin resistance, which implies impairment of insulin signaling in the target tissues, is a common cause of type 2 diabetes. Adipose tissue plays an important role in insulin resistance through the dysregulated production and secretion of adipose-derived proteins, including tumor necrosis factor-alpha, plasminogen activator inhibitor-1, leptin, resistin, angiotensinogen, and adiponectin. Adiponectin was estimated to be a protective adipocytokine against atherosclerosis, and also to have an anti-inflammatory effect. In this study, the relationship between fasting plasma adiponectin concentration and adiposity, body composition, insulin sensitivity (ITT, HOMAIR, QUICK), lipid profile, fasting insulin concentration were examined in Korean type 2 diabetes. The difference in the adiponectin concentrations was also examined in diabetic and non-diabetic subjects, with adjustment for gender, age and body mass index. 102 type 2 diabetics and 50 controls were examined. After a 12-h overnight fast, all subjects underwent a 75gram oral glucose tolerance test. Baseline blood samples were drawn for the determinations of fasting plasma glucose, insulin, adiponectin, total cholesterol, triglyceride, LDL-cholesterol, and HDL-cholesterol. The body composition was estimated using a bioelectric impedance analyzer (Inbody 2.0). The insulin sensitivity was estimated using the insulin tolerance test (ITT), HOMAIR and QUICK methods. In the diabetic group, the fasting adiponectin concentrations were significantly lower in men than in women. They were negatively correlated with BMI (r=-0.453), hip circumference (r=-0.341), fasting glucose concentrations (r=-0.277) and HOMAIR (r=-0.233). In addition, they were positively correlated with systolic blood pressure (r=0.321) and HDL-cholesterol (r= 0.291). The systolic blood pressure and HDL-cholesterol were found to be independent variables, from a multiple logistic regression analysis, which influenced the adiponectin concentration. Compared with the non-diabetic group, the adiponectin concentrations were significantly lower in the diabetic group, with the exception of obese males. In conclusion, the plasma adiponectin concentrations were closely related to the insulin resistance parameters in Korean type 2 diabetic patients.
Adult ; Aged ; Biological Markers ; Diabetes Mellitus, Type 2/*blood ; Female ; Humans ; *Insulin Resistance ; Intercellular Signaling Peptides and Proteins/*blood ; Korea ; Male ; Middle Aged

BACKGROUND: QT dispersion, reflecting inhomogenous ventricular repolarization, increases in myocardial ischemia. In addition, Many studies reported that prolonged QT dispersion reduced to normal after reperfusion treatment. We have carried out this study to evaluate the QT and JT dispersion before and after the angioplasty in patients with coronary artery diseases. METHOD AND MATERIALS: Seventy-two patients (55 men and 17 women:18 acute myocardial infarction, 9 unstable angina and 45 stable angina) who underwent percutaneous transluminal coronary angioplasty were evaluated. Standard 12-lead electrocardiograms were recorded 24 hours before and 24 hours after angioplasty at a paper speed of 25 mm/sec. RESULTS: There was no significant change in heart rate or the maximum or minimum QT interval after angioplasty. QT dispersion significantly decreased after angioplasty (p<0.05). And QTc dispersion (QTcd) also similary reduced (p<0.01). JT dispersion (JTc) and JTc dispersion (JTcd) were not changed significantly. There were significant reduction in QTd and QTcd in the group of patients without acute myocardial infarction (p=0.005, 0.004, respectively) but not in JTd and JTcd. However, in patients with acute myocardial infarction, all four dispersion were not reduced significantly. And in patients with multivessel angioplasty, there were significant reduction of QTd, QTcd, JTd and JTcd (p=0.016, 0.014, 0.036, 0.030, respectively). CONCLUSIONS: As changes in QT and JT dispersion reflect successful reperfusion by angioplasty, they can be accepted as promising test for assessing the effectiveness of angioplasty clinically. However the methodology still has several unresolved issues and larger, prospective clinical studies are needed.
Angina, Unstable ; Angioplasty* ; Angioplasty, Balloon, Coronary ; Coronary Artery Disease ; Electrocardiography ; Heart Rate ; Humans ; Male ; Myocardial Infarction ; Myocardial Ischemia ; Reperfusion

Crohn's disease is a rare inflammatory bowel disease in Korea which requires a differential diagnosis with intestinal tuberculosis. In the early stages of Crohn's diseaae, the combiation of bowel wall edema and spasm produces intermittent obstructive manifestations, so the usual subjective symptoms were appeared long standing diarrhea, low grade fever and abdominal pain and distension. We recently experienced a case of Crohn's colitis with repeated bowel obstruction in female adult who underwent surgical resection.
Abdominal Pain ; Adult ; Colitis ; Crohn Disease* ; Diagnosis, Differential ; Diarrhea ; Edema ; Female ; Fever ; Humans ; Inflammatory Bowel Diseases ; Korea ; Spasm ; Tuberculosis

Crohn's disease is a rare inflammatory bowel disease in Korea which requires a differential diagnosis with intestinal tuberculosis. In the early stages of Crohn's diseaae, the combiation of bowel wall edema and spasm produces intermittent obstructive manifestations, so the usual subjective symptoms were appeared long standing diarrhea, low grade fever and abdominal pain and distension. We recently experienced a case of Crohn's colitis with repeated bowel obstruction in female adult who underwent surgical resection.
Abdominal Pain ; Adult ; Colitis ; Crohn Disease* ; Diagnosis, Differential ; Diarrhea ; Edema ; Female ; Fever ; Humans ; Inflammatory Bowel Diseases ; Korea ; Spasm ; Tuberculosis

Pneumatosis intestinalis is an uncommon disorder characterized by an accumulation of gas in the bowel wall. We described three cases undertaking liver transplantation. The patients developed diarrhea in three cases and high fever in two. An abdominal X-ray and computed tomography scan demonstrated extensive pneumatosis intestinalis in the colon with pneumoperitoneum mimicking hollow organ perforation. However, the patients had no abdominal symptoms and there was no evidence of peritonitis. The infection work-up was negative except one case with cytomegalovirus antigenemia. After one week of conservative management including bowel rest and antibiotic therapy, their pneumoperitoneum resolved spontaneously without any complication. Pneumatosis intestinalis should be considered as a differential diagnosis after adult liver transplantation with patients suffering from watery diarrhea and fever. Pneumoperitoneum, air-density in mesentery and retroperitoneum in patients with pneumatosis intestinalis without signs of peritonitis improved with conservative management, which included bowel rest and antibiotic therapy.
Adult ; Colon ; Cytomegalovirus ; Diagnosis, Differential ; Diarrhea ; Fever ; Humans ; Liver ; Liver Transplantation ; Mesentery ; Mortuary Practice ; Peritonitis ; Pneumoperitoneum ; Stress, Psychological

CT and portographic findings of 63 patients with hepatoma, undergone hepatic angiography and superiormesenteric portography for evaluation of tumor and thrombosis of portal vein and determination of indication oftranscatheter arterial embolization for palliative treatment of hepatoma from April,85 to June, 86 in Hanyanguniversity hospital, were reviewed. The results were as follows: 1. In 36 cases, portal vein thrombosis wasdetected during portography. Nineteen of 37 cases which revealed localized hepatoma in the right lobe of the livershowed portal vein thrombosis; 9 of 11 cases of the left lobe; 8 of 14 cases which were involved in entire liverrevealed thrombosis. One case localized in the caudate lobe showed no evidence of invasion to portal vein. 2.Twenty-four of 34 cases with diffuse infiltrative hepatoma revealed portal vein thrombosis and the incidence ofportal vein thrombosis in this type were higher than in the cases of the nodular type. 3. The portal veinthrombosis appeared as filling defects of low density in the lumen of the portal veins in CT and they did notreveal contrast enhancement. 4. CT revealed well the evidences of obstructions in the cases of portal veinthrombosis and the findings were well-corresponded to the findings of the superior mesenteric portography. 5. Fiveof the cases of the portal vein thrombosis were missed in the CT and the casuses were considered as due to partialvolume effect of enhanced portal vein with partial occlusion or arterioportal shunts. 6. Six of 13 cases withocclusion of main portal vein showed cavernous transformation and they were noted as multiple small enhancedvascularities around the porta hepatis in the CT. According to the results, we conclude that CT is a usefulmodality to detect the changes of the portal veins in the patients of the hepatoma.
Angiography ; Carcinoma, Hepatocellular ; Humans ; Incidence ; Palliative Care ; Portal Vein ; Portography ; Thrombosis ; Veins ; Venous Thrombosis

PURPOSE: Leptin may play an important role in the pathophysiology of osteoarthritis. However, the effect of letpin on the anabolic and catabolic metabolisms in chondrocytes remains unclearly elucidated. Therefore, the purpose of this study was to investigate the effect of leptin on proliferation, anabolic and catabolic metabolism of chondrocyte using ATDC5 chondrogenic cell line. MATERIALS AND METHODS: The effects of leptin on chodnrocyte proliferation, anabolic and catabolic meatabolism were examined in ATDC5 cells treated with leptin at varying concentrations(10, 100, 300, 600 ng/ml) for 24, 48, and 72 hours. The cell proliferation was evaluated by MTT assay. The anabolic and catabolic activities were assayed by RT-PCR for transforming growth factor-beta(TNF-alpha), proteoglycan-4 (PRG4), type- I collagen (type- I Col) and tumor necrosis factor-beta(TNF-alpha), matrix metalloproteinase -2 (MMP-2), respectively. RESULTS: Leptin treatment did not influence cell proliferation of chondrocyte regardless of concentration. TGF-beta expression was increased until 48 hours of leptin treatment compared to controls. Especially, it was significantly increased in leptin of 10 ng/ml and 100 ng/ml (P<0.05). PRG4 expression was not different between letpin treatment and controls. Type-I Col expression was decreased in dose- and time-dependent manner. Leptin of 10ng/ml significantly inhibited MMP-2 and TNF-alpha expressions compared to controls (P<0.05). CONCLUSION: This study shows that leptin at low concentration increases TGF-beta expression, but inhibits the expression of TNF-alpha and MMP-2. Also this study shows that leptin do not affect the cell proliferation of chondrocytes. These results suggest that leptin at low or physiological level contributes to the prevention of cartilage damage by stimulating anabolic activity and inhibiting catabolic activity of chondrocyte rather than chondrocyte regeneration by increasing cell proliferation.
Cartilage ; Cell Proliferation ; Chondrocytes ; Collagen ; Leptin ; Necrosis ; Osteoarthritis ; Regeneration ; Transforming Growth Factor beta ; Tumor Necrosis Factor-alpha

A 67-year-old woman was admitted to our hospital under suspicion of a hepatic tumor, which had been previously diagnosed to be an adenocarcinoma by fine needle aspiration. Computed tomography and magnetic resonance imaging revealed a large tumor measuring 9 cm in diameter in Couinaud's hepatic segments 4, 5, and 8. We diagnosed the patient to have primary liver cancer, and suspected intrahepatic cholangiocarcinoma preoperatively. We performed a central hepatectomy. According to the immunohistochemical findings of the resected specimen, the tumor was diagnosed to be a primary neuroendocrine carcinoma in the liver. The patient is presently alive without recurrence at 3 months after hepatic resection.
Adenocarcinoma ; Biopsy, Fine-Needle ; Carcinoma, Neuroendocrine ; Cholangiocarcinoma ; Chromogranin A ; Female ; Hepatectomy ; Humans ; Liver ; Liver Neoplasms ; Magnetic Resonance Imaging ; Recurrence

BACKGROUND: While the number of deceased donor donations has increased in Korea, the organ shortage remains a major limitation for kidney transplantation. Donation after circulatory death (DCD) can be an option to expand the donor pool. In this study we evaluated the short and long term survival of grafts and patients and assessed the risk factors for graft failure. METHODS: In a single center, from August 1997 to December 2013, 28 cases of recipients who received kidney transplantation from DCD were enrolled. Information about donor and recipient factors, graft conditions, and transplant outcomes was collected through review of medical records. We calculated overall graft and patient survival rates and the risk factors for graft failure according to donor criteria and whether or not delayed graft function (DGF) occurred. RESULTS: There was no primary non-function, but DGF developed in 67.9% (19/28). Graft losses occurred in five patients during a median follow-up period of 68.2 months (4~204). There was no significant difference in graft survival rates depending on the donor criteria and the occurrence of DGF. In addition, there were no noteworthy risk factors for graft failure among donor age, donor creatinine, extended criteria donor, recipient age, warm ischemic time, cold ischemic time, and DGF. CONCLUSIONS: In this study, despite the high incidence of DGF, the long-term graft and patient survival in kidney transplantation from DCD were acceptable. Therefore, DCD can be an alternative to expand the donor pool and to shorten the waiting time.
Brain Death* ; Brain* ; Cold Ischemia ; Creatinine ; Delayed Graft Function ; Follow-Up Studies ; Graft Survival ; Humans ; Incidence ; Kidney Transplantation* ; Kidney* ; Korea* ; Medical Records ; Risk Factors ; Survival Rate ; Tissue Donors* ; Transplants ; Warm Ischemia