Primary biliary cirrhosis(PBC) is a chronic cholestatic liver disease of unknown origin. The small and medium sized intrahepatic bile ducts are destroyed by an inflammatory process, which, it has been suggested, is of the autoimmune type. It is strongly associated with the presence of antimitochondrial antibodies, predominantly IgM and IgG. The liver changes are classified into four stages, of which stage IV represents the development of cirrhosis, which required orthotropic liver transplantation in the longrun. The prevalence rates was reported 128 per millon in Sweden , but the disease is relatively rare in Oriental area. In medical treatment, long-term administration of ursodeoxycholic acid improves both clinical and biochemical signs, slows the progression of the disease and reduces the complication requiring liver transplantation. We report two cases of PBC, one with histologically proven cirrhosis, and the other with bile duct destruction consistent with stage III and hypothyroidism.
Antibodies ; Bile Ducts ; Bile Ducts, Intrahepatic ; Fibrosis ; Hypothyroidism ; Immunoglobulin G ; Immunoglobulin M ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Liver Transplantation ; Prevalence ; Sweden ; Ursodeoxycholic Acid

No abstract available.

No abstract available.
Diagnosis* ; Thrombosis*

PURPOSE: To speculate the role of bcl-2 protooncogene in endometrial carcinogenesis by determination of the expression of bcl-2 in endometrial hyperplasia and carcinoma. MATERIALS AND METHODS: We studied bcl-2 expression by an immunohistochemical method in the paraffin-embedded blocks of 78 patients with endometrial hyperplasia, 64 with simple hyperpasia, 9 with complex hyperplasia and 5 with atypical hyperplasia respectively, and 33 endometrial carcinoma treated at Asan Medical Center from June, 1989 to May, 1997. Intensity of bcl-2 staining was scored on a scale of 0 to 4, calibrated by comparison with stromal lymphocytes, which always received a score of 4. RESULTS: The results of this study showed that bcl-2 was relatively highly expressed in simple (n= 64), complex (n=9) and atypical hyperplasias (n=5) with mean staining scores of 2.95+/-1.09 (Mean+Standard Deviation), 2.78+/-1.20 and 3.60+/-0.89 respectively, which showed no difference among histologic types. In endometrial carcinoma, the expression of bcl-2 was significantly down regulated (mean score=1.76+/-1.35) compared with that of hyperplasia, and did not conelate with FIGO surgical stage. However, grade III tumor showed significantly lower expression that grade I or II tumor. CONCLUSION: Bcl-2 expression is down regulated in endometrial carcinoma than endo- metrial hyperplasia, and correlates with tumor grade, which suggest that bcl-2 expression might be the result of carcinogenesis or bcl-2 plays only an adjunctive role in the endometrial carcinogenesis.
Carcinogenesis ; Chungcheongnam-do ; Endometrial Hyperplasia* ; Endometrial Neoplasms ; Female ; Humans ; Hyperplasia ; Lymphocytes

No abstract available.
Thoracic Surgery*

No abstract available.
Fontan Procedure*

Understanding of the surgical anatomy of patients with double outlet right ventricle (DORV) is important in the planning of biventricular repair. From May 1995 to September 1996, 7 patients underwent biventricular repair for DORV with remote ventricular septal defect. There were 5 males and 2 females. Age at operation varied from 2 to 9 years(mean 3.4+/-2.7years). Preoperative diagnostic assessment was made by two-dimensional echocardiography and cardiac catheterization. Ventricular septal defect was perimembranous inlet type in all patients. Associated cardiac anomalies were pulmonary atresia in two, pulmonary stenosis in five and tricuspid chordae attachment to conal septum in five. The operations were performed intraventricular repair and pulmonary enlargement in two, REV operation in two, and Rastelli operation in three. There was no early postoperative deaths and complications. The follow-up period was from 1 month to 18months, averaging 10+/-6.1 months. In the past,we considered the Fontan operation indicative as primary choice when DORV was associated with abnormal tricuspid chordal attachment to the conal septum,but now we believe that biventricular repair is feasible for those cases by making conal flap or reattachment method. Biventricular repair has theoretic advantages because it estabilishes normal anatomy and physiology,and it was concluded that the precise preoperative evaluation using both echocardiography and cardiac catheterization was essential to the successful surgery.
Bays ; Cardiac Catheterization ; Cardiac Catheters ; Double Outlet Right Ventricle* ; Echocardiography ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Septal Defects, Ventricular* ; Humans ; Male ; Pulmonary Atresia ; Pulmonary Valve Stenosis

BACKGROUND/AIMS: Because there is a lack of effective biomarkers, we aimed to discover proteomic candidate markers for hepatocellular carcinoma (HCC) in cirrhotic patients at the highest-risk of HCC, and to validate the markers. METHODS: We collected tumor tissue from 5 cirrhotics with HCC, and from 5 cirrhotics without HCC, who underwent liver resection or transplantation. These tissue samples were analyzed by 2-dimensional difference gel electrophoresis coupled with matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS), and potential markers were validated at the transcriptional and translational levels. We also performed western blot assays using other blood samples from 10 cirrhotics with HCC and 10 without HCC. RESULTS: Among the 66 distinguishable spots on 2-D gel images, we identified 15 proteins overexpressed more than 1.5 fold in terms of volume ratio in the tumors. Ten of the over-expressed proteins were identified by MALDI-TOF MS; of those, only methionine adenosyltransferase 1 (MAT1), a protein specific for liver, and acyl-CoA dehydrogenase were significantly up-regulated in tumors in further immunoblotting analyses (Ps<0.05). There was no between-pair difference in MAT1 mRNA measured by real-time polymerase chain reaction (P=0.96). However, in western blots of serum samples, distinct MAT1 bands were observed in all 10 HCC patients, but in only 2 of the non-HCC patients. CONCLUSIONS: MAT1 is a potential marker for surveillance in cirrhotic patients with and without prior HCC.
Acyl-CoA Dehydrogenase ; Biomarkers ; Blotting, Western ; Carcinoma, Hepatocellular ; Humans ; Immunoblotting ; Liver ; Liver Cirrhosis ; Mass Spectrometry ; Methionine Adenosyltransferase ; Methionine ; Proteomics ; Real-Time Polymerase Chain Reaction ; RNA, Messenger ; Two-Dimensional Difference Gel Electrophoresis