1/131. Primary biliary cirrhosis associated with membranous glomerulonephritis.A 33-year-old woman was admitted to our department for evaluation of liver dysfunction and proteinuria. A liver biopsy specimen showed ductular proliferation and moderate portal fibrosis indicating stage II primary biliary cirrhosis. A renal biopsy specimen showed mild to moderate mesangial cell proliferation without crescent formation or interstitial nephritis. Immunofluorescent staining revealed deposition of immunoglobulin g (IgG), third component of complement (C3), and Clq on glomerular basement membranes. The findings indicated stage I membranous glomerulonephritis. Administration of ursodesoxycholic acid together with prednisolone, azathioprine, and dipyridamole decreased proteinuria and improved cholestatic liver dysfunction.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
2/131. Negative conversion of antimitochondrial antibody in primary biliary cirrhosis: a case of autoimmune cholangitis.Autoimmune cholangitis is a clinical constellation of chronic cholestasis, histological changes of chronic nonsuppurative cholangitis and the presence of autoantibodies other than antimitochondrial antibody (AMA). It is uncertain whether this entity is definitely different from AMA positive primary biliary cirrhosis (PBC), though it shows some differences. We report a case of autoimmune cholangitis in a 59-year-old woman, who had been previously diagnosed as AMA-positive PBC associated with rheumatoid arthritis, has been converted to an AMA-negative and anticentromere antibody-positive PBC during follow-up. The response to ursodeoxycholic acid treatment is poor except within the first few months, but prednisolone was dropping the biochemical laboratory data.- - - - - - - - - - ranking = 330.14060633993keywords = cholestasis (Clic here for more details about this article) |
3/131. Primary biliary cirrhosis associated with painless thyroiditis.A case of anti-mitochondrial antibody (AMA)-negative primary biliary cirrhosis (PBC) associated with painless thyroiditis is reported in a 47-year-old woman who diagnosed as PBC based on her elevated serum gamma-glutamyl transpeptidase and immunoglobulin m levels, as well as histological findings of destroyed bile ducts surrounded by mononuclear infiltrates in the biopsied liver. She was negative for AMA and had a depressed level of thyroid-stimulating hormone accompanied by increased free thyrosine, thyroxine and triiodothyronine levels and low titers of anti-microsomal and anti-thyroid peroxidase antibodies. Her thyroid disorder corresponded with painless thyroiditis. An association between PBC and hyperthyroidism is rare. Furthermore, an association between AMA-negative PBC and hyperthyroidism due to painless thyroiditis has not previously been reported.- - - - - - - - - - ranking = 26.713646455697keywords = bile duct, bile, duct (Clic here for more details about this article) |
4/131. Case report: a rare association of primary biliary cirrhosis and systemic lupus erythematosus and review of the literature.We report a rare occurrence of systemic lupus erythematosus in a patient known to have had well-documented primary biliary cirrhosis for 10 years. The presentation was dramatic with pericardial tamponade, but responded well to high dose corticosteroid. There are only five such definite associations reported in the literature. In the present case, other possible causes were considered, such as drug-induced cholestasis, drug-induced lupus, autoimmune chronic active hepatitis and the overlap syndrome.- - - - - - - - - - ranking = 330.14060633993keywords = cholestasis (Clic here for more details about this article) |
5/131. hepatitis c virus-related fibrosing cholestatic hepatitis after cardiac transplantation: is azathioprine a contributory factor?We report a patient who acquired hepatitis c virus (HCV) infection at cardiac transplantation, developing fibrosing cholestatic hepatitis (FCH) with early liver failure and a fatal outcome. FCH is a recently described clinicopathological entity characterized by a cholestatic pattern of serum liver enzyme abnormalities, a progressive course leading to liver failure, and a pathological picture defined by periportal fibrosis, neutrophilic infiltrates and signs of histological cholestasis. Although it was initially described secondary to hepatitis b virus infection, it has also been recently related to HCV infection. Some histopathological features consistent with azathioprine hepatotoxicity like cholestasis, perisinusoidal fibrosis, veno-subocclusive lesions and nodular regenerative hyperplasia were also observed in this case. Therefore, a direct cytopathic effect of HCV and the concurrent pathogenic role of azathioprine hepatotoxicity may be involved in the development of this complication of transplantation.- - - - - - - - - - ranking = 660.28121267986keywords = cholestasis (Clic here for more details about this article) |
6/131. Autoimmune cholangitis: case report.We report on 2 patients who showed mixed signs of primary biliary cirrhosis and autoimmune hepatitis. Both patients were female, in their fifties (54 and 58), their laboratory tests indicated cholestasis, and a liver biopsy revealed liver cirrhosis with significant lesions of the bile ducts. Both were treated with prednisolone with their liver tests showing a rapid normalization of their aminotransferases. These patients can be considered as presenting with what is known as the overlap syndrome or autoimmune cholangitis, which has the clinical, biochemical, immunological, and histopathological characteristics of primary biliary cirrhosis and autoimmune hepatitis type I.- - - - - - - - - - ranking = 356.85425279563keywords = cholestasis, bile duct, bile, duct (Clic here for more details about this article) |
7/131. Primary biliary cirrhosis associated with type A gastritis and chronic thyroiditis.We report a patient with primary biliary cirrhosis (PBC) associated with type A gastritis, chronic thyroiditis, and iron deficiency anemia. The patient was a 45-year-old Japanese woman who was admitted to our hospital with severe microcytic and hypochromic anemia, abnormal results for liver function tests, and a diffuse goiter. The diagnosis of PBC (Scheuer's stage II) was confirmed by the presence of specific anti-mitochondrial antibody in high titers and histological examination showing chronic non-suppurative destructive cholangitis with bridging fibrosis. Additionally, marked atrophic mucosa throughout the body and fundus of the stomach was observed endoscopically, and there was positivity for intrinsic factor antibody, an extremely low ratio of serum pepsinogen a to C, and hypergastrinemia, indicating coexisting type A gastritis. The severe anemia was thought to be caused by failure of dietary iron absorption related to achlorhydria with this gastritis. However, the serum level of vitamin B12 was normal. She also had autoimmune thyroiditis. PBC is frequently associated with extrahepatic autoimmune diseases, including ductular lesions. However, the association of PBC with type A gastritis is quite rare, although the stomach is also an exocrine glandular structure. This particular case, in addition to previous reports, leads to a discussion of whether type A gastritis should be regarded as a possible, although uncommon, component disorder of so-called dry gland syndrome.- - - - - - - - - - ranking = 95.865059541926keywords = extrahepatic, duct (Clic here for more details about this article) |
8/131. hemobilia, a rare cause of acute pancreatitis after percutaneous liver biopsy: diagnosis and treatment by endoscopic retrograde cholangiopancreatography.We here report the case history of a 75-yr-old woman who developed pancreatitis and recurrent symptomatic, cholestasis-induced hemobilia after percutaneous liver biopsy. An endoscopic sphincterotomy with clot extraction led to relief of symptoms. The risk of hemobilia after percutaneous liver biopsy is less than one per 1000 procedures, and only two cases of acute pancreatitis after percutaneous liver biopsy have previously been reported. To our knowledge, this is the first case in which endoscopic retrograde cholangiopancreatography was used to both diagnostic and therapeutic ends.- - - - - - - - - - ranking = 330.14060633993keywords = cholestasis (Clic here for more details about this article) |
9/131. Superior vena cava obstruction and liver transplantation in a child.We report a case of superior vena cava obstruction in a child, which was probably secondary to long-term central venous cannulation. The obstruction was asymptomatic preoperatively, but became evident during liver transplantation, and complicated the intraoperative management. There is one other case report of this occurring in an adult in similar circumstances, and we believe that ours is the first report of such a presentation in the paediatric age group.- - - - - - - - - - ranking = 0.35705509404377keywords = obstruction (Clic here for more details about this article) |
10/131. Primary hepatic marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type in a patient with primary biliary cirrhosis.Primary lymphoma of the liver is rare. Recently, marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) type have been described in the liver. Most of these cases occurred without known underlying liver disease, while others were seen in patients with chronic hepatitis. A case of primary hepatic MALT lymphoma in a patient with primary biliary cirrhosis was reported recently. Some authors have proposed that chronic persistent immunogenic stimulation causes development of acquired MALT and subsequently MALT lymphoma, based on the observation of MALT lymphoma in association with infectious agents, such as helicobacter pylori and hepatitis c virus, and autoimmune diseases, such as Hashimoto thyroiditis and Sjogren syndrome. Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease characterized by destruction of intrahepatic small to medium-sized bile ducts; this disease is mediated by a cytotoxic T-cell reaction. The prolonged immune activation in primary biliary cirrhosis may play a role in the lymphomagenesis of hepatic MALT lymphoma. We describe another case of primary hepatic MALT lymphoma, which was found incidentally in a patient with end-stage primary biliary cirrhosis. This case further supports the role of immunogenic stimulation in the pathogenesis of this particular low-grade B-cell lymphoma.- - - - - - - - - - ranking = 26.713646455697keywords = bile duct, bile, duct (Clic here for more details about this article) |
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