1/174. Acquired inhibitors to the coagulation factor xii associated with liver disease.Three patients with liver disease and prolonged activated partial thromboplastine time (APTT) on routine tests are presented. One woman had metastatic liver disease from gastric carcinoma, a second woman had autoimmune hepatitis, and one man had severe chronic hepatitis b. APTT was not corrected after mixing experiments with 25%, 50%, and 75% of normal pool plasma, indicating the presence of an acquired inhibitor. In all three cases, factor xii coagulant activity was reduced: <1%, <1%, and 3%, respectively, while all of the other coagulation factors were normal. In all three cases no other auto-antibody was detected. In the first patient, APTT was normalized after a left liver lobectomy, whereas the primary lesion remained unresected. In the second patient, the FXII activity was improved after corticosteroid therapy but never returned to normal values. In the third patient, the APTT was improved after hydroxychloroquine therapy. None of the patients had hemorrhagic or thrombotic phenomena.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/174. The combined occurrence of macular amyloidosis and prurigo nodularis.The association of prurigo nodularis (PN) and macular amyloidosis (MA) has not been reported before. Although pruritus related frictional trauma is a well-known cause of PN, its role in the development of MA has always been questioned. We herein report two cases with chronic liver disease and iron deficiency who concomitantly developed MA and PN lesions. pruritus was the preceding factor and both lesions were confined to scratched areas. The association of two otherwise uncommon dermatoses in pruritic patients and their characteristic distribution might indicate an important role for pruritus-induced scratching in the pathogenesis of MA, too.- - - - - - - - - - ranking = 0.14285714285714keywords = liver (Clic here for more details about this article) |
3/174. Progressive portopulmonary hypertension after liver transplantation treated with epoprostenol.Portopulmonary hypertension (PPHTN) is an uncommon complication of advanced liver disease. epoprostenol has been effective in the treatment of PPHTN and has been used as a bridge to orthotopic liver transplantation (OLT). The role of OLT in the reversal of PPHTN is unclear. We report a case of severe PPHTN (mean pulmonary artery pressure of 45 mm Hg) that progressed after OLT. Acute dosing with epoprostenol improved the pulmonary vascular resistance by 55% and the cardiac index by 134%. Hemodynamic and symptomatic improvements were maintained after 18 months of long-term treatment with epoprostenol. This is the first reported case of a successful favorable outcome after treatment for progressive PPHTN after OLT. Our case report complements previous reports by highlighting the potential effective use of epoprostenol as a definitive treatment for PPHTN.- - - - - - - - - - ranking = 0.85714285714286keywords = liver (Clic here for more details about this article) |
4/174. Cytoplasmic inclusion bodies and minimal hepatitis: fibrinogen storage without hypofibrinogenemia.A 12-year-old Japanese boy had chronic elevation and fluctuation of serum transaminase levels since infancy, with no signs or symptoms of liver failure. Usual infections or metabolic disorders were eliminated from consideration. No coagulopathy or abnormality in plasma concentrations of clotting factors was found. light microscopy of liver biopsy specimens obtained at ages 2, 5, and 7 years showed slight hepatocyte disarray and minimal mononuclear-leukocyte lobular inflammation, with eosinophilic inclusion bodies in the cytoplasm of hepatocytes throughout the lobule. These bodies stained with the periodic acid-Schiff (PAS) technique; the PAS-positive material was partly diastase digestible and on immunostaining marked for fibrinogen but not for alpha 1-antitrypsin. On transmission electron microscopy, the bodies were represented by finely granular material contained within membranes and were interpreted as tentatively endoplasmic reticulum. fibrinogen storage may be manifest as minimal hepatitis without coagulopathy.- - - - - - - - - - ranking = 0.28571428571429keywords = liver (Clic here for more details about this article) |
5/174. A case of autoimmune hepatitis with a high titer of antimitochondrial antibody and normal gamma-globulinemia.We report here a patient with chronic active hepatitis who had no markers for hepatitis viruses and no hyper-gamma-globulinemia, but had high titers of antimitochondrial antibody. serum levels of alkaline phosphatase were normal, and antinuclear antibody, antismooth muscle antibody, and antiliver kidney microsome antibody tested negative. The titers of antimitochondrial antibody exceeded 1:640, and the positivity for anti-M2 was ascertained by using both ELISA and immunoblot with beef-heart mitochondria and a recombinant pyruvate dehydrogenase E2 subunit as antigens. This patient responded to ursodeoxycholic acid (UDCA) therapy in the beginning, but her hepatitis flared up during UDCA therapy. In contrast, she responded completely to corticosteroid therapy. The clinical course and histological findings of this patient strongly suggest that this patient has autoimmune hepatitis.- - - - - - - - - - ranking = 0.14285714285714keywords = liver (Clic here for more details about this article) |
6/174. Successfully resected hepatoblastoma in a young adult with chronic hepatitis b: report of a case.hepatoblastoma usually occurs in children, but a few cases have also been reported in adults. We report the unusual case of hepatoblastoma in an 18-year-old adult with chronic hepatitis b. He visited a local hospital with right upper abdominal pain. Abdominal ultrasound showed a large mass in the right lobe of his liver. He was referred to our hospital and admitted for further examination. At admission, liver function tests gave slightly elevated results (aspartate aminotransferase (AST) 103 IU/l, alanine aminotransferase (ALT) 63 IU/l). A test for hepatitis virus revealed that he was a hepatitis b surface antigen (HBsAg) carrier and had experienced seroconversion. His alpha-fetoprotein (AFP) was elevated to 1 548 000 IU/ml. Abdominal ultrasound showed a 109 x 96 x 80-mm mass with mosaic pattern in the right lobe of the liver and right portal vein thrombus. Abdominal computed tomography (CT) demonstrated a large low-density mass occupying the right lobe, with some high-density parts that showed calcification. From these results, we diagnosed hepatoblastoma in a young adult. A right lobectomy was performed. Pathological examination showed a highly differentiated hepatoblastoma. Adjuvant chemotherapy was performed with cisplatin and pirarubicin. The patient has been well and free of recurrence for 12 months, and his AFP level remains almost normal.- - - - - - - - - - ranking = 0.42857142857143keywords = liver (Clic here for more details about this article) |
7/174. Liver fibrosis attributed to lipid lowering medications: two cases.We identified two cases of chronic active hepatitis with liver fibrosis induced by lipid lowering drugs of the statin and fibrate classes despite regular monitoring of transaminases. There are few reports of clinically significant hepatitis induced by these drugs and even fewer cases of fibrosis. Given the growing use of these drugs, there are implications for monitoring patients on long-term therapy for liver damage.- - - - - - - - - - ranking = 0.28571428571429keywords = liver (Clic here for more details about this article) |
8/174. hepatitis e virus superinfection in patients with chronic liver disease.Infection with hepatitis a virus (HAV) can cause severe illness in adult patients with chronic liver disease (CLD) caused by hepatitis c. In endemic areas such as South asia, however, most adult patients already have been exposed to HAV but could still be susceptible to hepatitis e virus (HEV) infection. We document that HEV superinfection in 4 of our CLD patients caused severe liver decompensation. We then determined the seroprevalence of HAV and HEV in 233 patients with stable CLD, with the goal of defining the need for protection against these viruses in these patients. overall, 41 (17.5%) of 233 CLD patients were HEV antibody immunoglobulin g (IgG)-positive, and 228 of 233 (97.8%) were HAV IgG-positive. As controls, we tested 90 age- and sex-matched healthy volunteer blood donors for HAV and HEV antibodies IgG. There was no difference in the percentage of CLD patients and blood donors positive for HEV antibody IgG (17.7% vs. 17.5%) or for HAV IgG (97.8% vs. 94%). No differences were observed in the severity of liver disease between previously HEV-exposed and -nonexposed patients. In conclusion, superinfection with HEV in patients with underlying CLD can cause severe hepatic decompensation leading to increased morbidity and mortality. The large majority of adult CLD patients in endemic countries are vulnerable to infection with HEV, but are protected against hepatitis A, and are ideal candidates for an HEV vaccine.- - - - - - - - - - ranking = 1.0612597114837keywords = liver, hepatic (Clic here for more details about this article) |
9/174. glomerulonephritis with microtubular deposits associated with cryoglobulinemia and chronic active hepatitis.A 65-year-old-woman presented with edema, ascites, proteinuria and abnormal liver function tests. A small amount of mixed cryoglobulin was detected in her serum. Liver biopsy revealed mild chronic active hepatitis, but tests for hepatotropic viral infection were negative. Electron microscopy of the renal biopsy revealed glomerular electron-dense deposits that contained numerous tubular structures. Renal amyloidosis and light chain deposition disease were ruled out by appropriate histological techniques. The ultrastructural findings of renal biopsy suggested either cryoglobulinemic glomerulonephritis or immunotactoid glomerulopathy. Although the exact interrelationship among the peculiar glomerulopathy, cryoglobulinemia and chronic active hepatitis in the present case remains undetermined, this report enlarges the spectrum of glomerulopathy characterized by extracellular deposition of microtubules.- - - - - - - - - - ranking = 0.14285714285714keywords = liver (Clic here for more details about this article) |
10/174. Acute q fever pericarditis followed by chronic hepatitis in a two-year-old girl.Acute coxiella burnetii infection is most commonly a mild and self-limiting disease with fever, pneumonia and hepatitis. endocarditis is the most frequent clinical presentation of chronic infection. We report a 2-year-old child with q fever who presented with acute pericarditis and cardiac tamponade and who developed a chronic hepatic infection.- - - - - - - - - - ranking = 0.061259711483674keywords = hepatic (Clic here for more details about this article) |
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