1/252. 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 = liver (Clic here for more details about this article) |
2/252. 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 = 0.33333333333333keywords = liver (Clic here for more details about this article) |
3/252. Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report.BACKGROUND: pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
4/252. Primary hepatic lymphoma associated with primary biliary cirrhosis.We report a case of primary hepatic lymphoma in a 55-yr-old female patient with primary biliary cirrhosis and sjogren's syndrome. On July 1994, a tumor measuring 11 mm in diameter was detected in the right lobe of the liver by abdominal ultrasonography. A needle biopsy specimen showed the lesion to contain small- and medium-sized lymphoid cells without obvious atypia, and a provisional diagnosis of pseudolymphoma was made. About 2 yr later, the tumor increased to 15 mm in diameter, necessitating a second needle biopsy. Histological and genetic examinations confirmed non-Hodgkin's lymphoma of diffuse, mixed small and large cell, B-cell type. However, the size of the tumor remained almost stable (16 mm in diameter) over a period of 7 months after diagnosis, without any treatment for lymphoma, indicating a low grade malignancy. We document hepatic lymphoma as an additional complication of primary biliary cirrhosis.- - - - - - - - - - ranking = 0.33333333333333keywords = liver (Clic here for more details about this article) |
5/252. Development of autoimmune hepatitis following liver transplantation for primary biliary cirrhosis.Two patients undergoing liver transplantation for classical end-stage primary biliary cirrhosis (PBC) are described, who went on to develop de novo autoimmune hepatitis (AIH) in the transplanted liver. The presentation, in both instances, was with malaise and lethargy. Markedly elevated serum transaminases were found, together with a raised serum IgG and/or globulin fraction and histological features on liver biopsy typical of AIH. Both cases had had changes in their immunosuppressive therapy before the onset of AIH episodes, and both rapidly responded to reinstitution of steroid therapy. The finding, in each case, of a coincidental multiple HLA class I allele match between the recipient and their liver donor suggests that HLA class I-restricted mechanisms may play an important role in the pathogenesis of AIH.- - - - - - - - - - ranking = 2.6666666666667keywords = liver (Clic here for more details about this article) |
6/252. 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 = 1keywords = liver (Clic here for more details about this article) |
7/252. 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 = 1keywords = liver (Clic here for more details about this article) |
8/252. 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 = 0.33333333333333keywords = liver (Clic here for more details about this article) |
9/252. Increased survival in advanced primary biliary cirrhosis patients with regular albumin infusions?Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease. Frequently, it can slowly progress to cirrhosis and, finally, death. liver transplantation is the only way to avoid this endpoint. For patients not accepted for liver transplantation, only symptomatic treatment remains. In advanced PBC, one of the main concerns is a decreasing albumin level. As albumin is an important protein in the human body, we wondered whether regular albumin infusions may result in a higher quality of life and prolong survival. In this case report we describe three patients who survived for a remarkably long time on this treatment regimen before finally succumbing to the sequela of advanced liver disease. Some features of albumin are mentioned which may help explain this phenomenon. We conclude that regular albumin infusions may be a means of decreasing morbidity and prolonging survival in a subgroup of patients with advanced PBC. However, prospective evaluation of regular albumin infusions in a group of PBC patients (e.g. those rejected for liver transplantation) seems mandatory.- - - - - - - - - - ranking = 1.3333333333333keywords = liver (Clic here for more details about this article) |
10/252. 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 = 2.3333333333333keywords = liver (Clic here for more details about this article) |
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