Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An African American male of West Indies descent was diagnosed to have elevated transferrin saturation, hyperferritinemia, severe iron deposition in hepatocytes, and hepatic cirrhosis at age 4. He was treated with serial phlebotomy to maintain a normal serum ferritin concentration thereafter. We evaluated him at age 23 and confirmed that he had normal serum ferritin levels, severe iron deposition in hepatocytes, hepatic cirrhosis, and portal hypertension. He did not have endocrinopathy, cardiomyopathy, or arthropathy. He was homozygous for the novel hemojuvelin (HJV) premature stop-codon mutation R54X (exon 3; c.160A-->T). He did not have either HFE C282Y, H63D, or S65C, or deleterious coding region mutations of SLC40A1, TFR2, or HAMP. His erythrocyte measures and hemoglobin electrophoresis were consistent with alpha-thalassemia trait. We conclude that homozygosity for HJV R54X accounts for his severe, early age-of-onset hemochromatosis; his phenotype was probably modified by serial phlebotomy therapy.
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PMID:Early age-of-onset iron overload and homozygosity for the novel hemojuvelin mutation HJV R54X (exon 3; c.160A-->T) in an African American male of West Indies descent. 1849 90

A 50-year-old woman with a 4-year history of type 2 diabetes history was treated with nateglinide (270 mg/day) and metformin hydrochloride (500 mg/day). The recipient was her 55-year-old husband whose diagnoses were liver cirrhosis with type C chronic hepatitis (Child-Pugh C, score, 10; Model for End-Stage Liver Disease: 15), hepatocellular carcinoma (solitary, 2 cm), and hepatic encephalopathy. Her body weight was 50 kg and body mass index 21.6 kg/m2. Laboratory examinations showed fasting blood glucose of 110 mg/dL and hemoglobin A1c (HbA1c) of 6.6% upon admission. Right liver lobectomy was performed of a 563-g graft. Operative time was 253 minutes and blood loss 50 mL. She was discharged at postoperative day 9 without any complications. We changed nateglinide and metformin hydrochloride to insulin aspart or human insulin after admission. Blood glucose level was strictly controlled using a sliding scale of insulin. She received regular glucose check-ups at our outpatient clinic after discharge. She stopped using insulin and returned to nateglinide and metformin hydrochloride on postoperative day 25. Her blood glucose level was 80 to 150 mg/dL and HbA1c was 5.8% at 5 months after surgery. This type 2 diabetic living liver donor showed good control of the postoperative glucose level without exacerbation or diabetic complications.
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PMID:Successful management of a type 2 diabetic donor in living-donor liver transplantation: a case report. 1892 79

This case report describes a 50-year-old female patient with liver cirrhosis presented with anemia. She was found to be suffering from gastric antral vascular ectasia (watermelon stomach) on upper gastrointestinal endoscopy. She underwent multiple sessions with Argon plasma coagulation, a non-contact thermal method of hemostasis for the management of watermelon stomach. After 3 sessions, the lesions disappeared and the hemoglobin increased by 2.4 gm/dl without any need of transfusion.
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PMID:Successful treatment of watermelon stomach / GAVE syndrome by using argon plasma coagulation. 1894 Jan 24

To investigate how liver disease alter the serum glycated proteins as markers of diabetic control, we studied serum GA, A1c and especially GA/A1c ratio in 255 patients having over 35IU/L in ALT(transaminase) compared with those of 829 type 2 diabetes mellitus (DM) in cross sectional manner. 255 patients with liver diseases were divided into 69 patients with biopsy proven liver cirrhosis (LC), 66 patients with chronic hepatitis(CH) and 120 patients with fatty liver(FL) diagnosed by abdominal echography. The mean GA/A1c ratio (+/-SD) was significantly higher (p<0.0001) in LC group(3.71+/-1.03) than the other groups (3.03+/-0.45 for CH, 3.05+/-0.42 for DM), while the mean GA/A1c ratio in FL group was significantly lower(2.74+/-0.31) (p<0.0001)) than that of DM groups. In LC group the GA/A1c ratio increased significantly depending upon serum albumin and/or platelet reductions. The GA/A1c ratio was significantly correlated with the other laboratory data such as serum albumin, cholinesterase, total cholesterol levels and weakly correlated with serum hemoglobin level. We also followed the serum levels of GA and A1c and the GA/A1c ratio during about 13 months (5 times blood collections) in 18 patients enrolled in this study. Resultantly the coefficient of variation of GA/A1c ratio was the smaller than the others(GA, A1c). The ROC curve of GA/A1c ratio for LC versus FL group was the most reliable between four groups and the cut-off value for LC versus FL was 2.94. Theses results suggest that GA/A1c ratio could be an useful marker for different diagnosis when facing patients with abnormal serum ALT level in a clinical setting.
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PMID:[Relationship between glycated albumin (GA) and glycated hemoglobin (A1c) in 255 patients with liver diseases using cross-sectional laboratory data]. 1897 54

Hepatitis C virus (HCV) infection may deteriorate quality of life. The relationship between HCV infection and quality of life in hemodialysis (HD) patients is unknown. The demographic characteristics, comorbidities, biochemical parameters, and Malnutrition-Inflammation Score (MIS) were recorded. Child-Pugh classification, Beck Depression Inventory, and SF-36 were performed. Liver histopathology was examined. Thirty-two patients (21 Child-Pugh-A, 11 Child-Pugh-B) were included. There was high-grade portal necroinflammatory activity in 14, high-grade lobular necroinflammatory activity in 26, and hepatic fibrosis in 19 patients. Three patients had cirrhosis. Patients with a high stage of liver fibrosis had lower social functioning scores than patient with a low stage of fibrosis (P= 0.011). The only correlation was between aspartate aminotransferase and the physical function subscale (r=-0.395, P= 0.025). None of the SF-36 scores differed between Child-Pugh A and B patients. Instead, most of the SF-36 subscale and summary scores were related with hemoglobin, albumin, MIS and Beck Depression Score. Quality of life in HCV-infected HD patients was independent of liver disease severity anchors, but was correlated with anemia, malnutrition, and depression.
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PMID:Quality of life is not related with liver disease severity but with anemia, malnutrition, and depression in HCV-infected hemodialysis patients. 1921 Feb 80

We report about our experience with combined en-bloc liver-pancreas transplantation in 14 patients with liver cirrhosis and insulin dependent type 2 diabetes mellitus. Exocrine drainage was achieved by duodeno-duodenostomy. Median posttransplant follow-up is currently 92.5 months. All patients were rendered independent from insulin therapy shortly after transplantation. Levels of glycosylated hemoglobin normalized in all recipients. Mean fasting C-peptide values increased from pretransplant 7.0+/-1.7 ng/mL to 10.5+/-2.9 ng/mL 3 months posttransplantation (P<0.001). One recipient (7.1%) developed recurrent exogenous insulin dependence 7 years after transplantation. Pancreas allograft rejection was confirmed by endoscopic biopsy of donor duodenum mucosa in two patients (14.3%). Calculated 5- and 7-year survival is currently at 64.3% and 64.3%, respectively. Our results indicate that combined en-bloc liver-pancreas transplantation using duodeno-duodenostomy is technically feasible and leads to excellent long-term control of glucose metabolism in patients with liver cirrhosis and insulin-dependent type 2 diabetes.
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PMID:Combined en-bloc liver-pancreas transplantation in patients with liver cirrhosis and insulin-dependent type 2 diabetes mellitus. 1930 91

A 53-year-old woman with liver cirrhosis and hepatocellular carcinoma underwent living donor liver transplantation. After transplantation, her hemoglobin and hematocrit levels decreased to 6.3 g/dl and 18.5%, respectively, during the course of 3 days. A contrast-enhanced abdominal computed axial tomography (CAT) scan showed a hemoperitoneum in the right perihepatic space with no evidence of abdominal wall hematoma or pseudoaneurysm formation. An angiogram of the deep circumflex iliac artery (DCIA) showed extravasation of contrast media along the surgical drain, which had been inserted during the transplantation procedure. Transcatheter embolization of the branches of the DCIA was successfully performed using N-butyl cyanoacrylate.
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PMID:Deep circumflex iliac artery-related hemoperitoneum formation after surgical drain placement: successful transcatheter embolization. 1944 69

Chronic hepatitis C virus (HCV) infection has been associated with a wide number of immunologic disorders, ranging from clinically silent laboratory abnormalities (eg, autoantibody positivity) to severe systemic diseases (eg, cryoglobulinemic vasculitis). Autoimmune hemolytic anemia (AIHA), due to the production of antibodies against erythrocyte membrane antigens, is an uncommon extrahepatic manifestation in the setting of chronic hepatitis C. Herein we have reported the case of a 57-year-old woman with decompensated HCV-related cirrhosis awaiting orthotopic liver transplantation (OLT) who experienced severe AIHA. After 1 month of treatment with prednisone (1 mg/kg body weight/d), there was no significant amelioration of anemia. Rituximab, an anti-CD20 monoclonal antibody that depletes B-lymphocytes reducing serum immunoglobulins, was initiated (375 mg/m(2) IV, weekly for 4 weeks) with a prompt, sustained increase in hemoglobin. The drug was well tolerated; it did not interfere with the course of the liver disease. Thirty-one months after rituximab therapy with resolution of AIHA, the patient successfully underwent OLT using immunosuppression with tacrolimus and low-dose steroids. The patient was discharged on postoperative day 36. No infectious event occurred in the postoperative period. At 18 months follow-up after OLT, there has been no infectious or hematological event. Our experience supported the safety of rituximab use in patients with advanced HCV-related liver disease before OLT.
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PMID:Orthotopic liver transplantation after successful treatment with anti-CD20 monoclonal antibody (rituximab) for severe steroid-resistant autoimmune hemolytic anemia: a case report. 1946 May 65

Knull phenotype completely lacks all Kell system antigens. Anti-Ku antibody is seen in immunized persons with Knull phenotype by transfusion or pregnancy. It can cause a fatal hemolytic transfusion reaction. A 66-yr-old male patient with liver cirrhosis visited emergency center due to acute bleeding. The patient was at hypovolemic shock status: his blood pressure was 80/50 mmHg, pulse rate was 110/min and hemoglobin level was 4.4 g/dL. Because of the presence of antibody against high incidence antigen, we could not find any compatible blood for the patient. Nevertheless, 4 units of packed RBCs had to be transfused. Moderate hemolytic transfusion reaction was developed after transfusion. At endoscopic examination, blood was spurting from gastric cardiac varix. Endoscopic histoacryl injection was tried, and bleeding was successfully controlled. After bleeding stopped, he was managed for anemia using steroid and other medical therapy instead of transfusion. His hemoglobin level was improved to 7.7 g/dL at the time of discharge. Later he has been proved to have a Knull phenotype, which is very rare, and anti-Ku antibody. This report is the first case of anti-Ku in a Knull phenotype person in Korea, who experienced a moderate hemolytic transfusion reaction.
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PMID:[A hemolytic transfusion reaction due to Anti-Ku antibody in a patient with Knull phenotype: the first case in Korea]. 1957 22

Biological assay of human liver material shows various concentrations of hemoglobin producing material in various diseased states. Cardiac congestion with red atrophy of the liver shows values (Table 21) somewhat below the human normal-94 per cent compared to 162 per cent. It is probable that this is due to atrophy and destruction of the liver cells in the centers of the liver lobules. We believe the remaining liver cells have a normal content of hemoglobin producing factors. Amyloid (tuberculosis) and fatty (alcoholic) degeneration show (Table 22) subnormal values-111 per cent compared to 162 per cent. If we allow for the "dilution" of liver cell weight by the amyloid and fat we observe normal values for the concentration of hemoglobin producing factors. Carcinoma growth in the liver whether primary or secondary shows net figures (Table 23) which are low-75 per cent compared to normal 162 per cent. It appears that cancer tissue, even tumor cells originating from hepatic epithelium, contains no hemoglobin producing factors. If we allow for the "dilution" of the remaining liver cells by the cancer tissue the figures are within normal limits. Cirrhosis and hepatitis (Table 24) with no signs of true hepatic insufficiency or severe hepatic injury but death supervening from secondary infection or hemorrhage will give average normal values -164 per cent compared to normal human of 162 per cent. Icterus is not a factor. Cirrhosis or hepatitis with severe parenchyma injury and signs of hepatic insufficiency give low values (Table 25) in biological assay -48 per cent compared to normal human 162 per cent. It may be surprising that the liver cells hold so tenaciously to these unknown factors influencing hemoglobin production but obviously only in severe liver injury is this concentration seriously reduced.
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PMID:HEMOGLOBIN PRODUCTION FACTORS IN THE HUMAN LIVER : II. LIVER DEGENERATION, CANCER, CIRRHOSIS AND HEPATIC INSUFFICIENCY. 1987 Jan 53


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