In recent years, autoimmune pancreatitis has been established as a special type of chronic pancreatitis. It is characterized by its histopathological and immunological features. The morphological hallmarks are periductal infiltration by lymphocytes and plasma cells and granulocytic epithelial lesions with consequent destruction of the duct epithelium and venulitis. Autoimmune pancreatitis has therefore also been called lymphoplasmacytic sclerosing pancreatitis, duct-destructive chronic pancreatitis, or sclerosing pancreatitis. Autoimmune pancreatitis most commonly involves the head of the pancreas and the distal bile duct. Occasionally, masses are formed and it has been described as an inflammatory myofibroblastic tumor. (+info)
A case of primary intrahepatic sclerosing cholangitis (PISC) complicated with atypical biliary epithelial proliferation.
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A 70-yr-old woman was admitted to our hospital with duodenal ulcer and anemia. The result of liver function test was abnormal and showed persistent elevated alkaline phosphatase levels. Thus, after recovery from duodenal ulcer, endoscopic retrograde cholangiopancreatography (ERCP) was performed; the characteristic "beaded" appearance with band-like strictures and saccular outpouchings affecting the intrahepatic biliary system were found. The diagnosis of primary intrahepatic sclerosing cholangitis (PISC) was made on the basis of the generally accepted diagnostic criteria of primary sclerosing cholangitis (PSC). However, the histological finding from a liver biopsy specimen revealed highly atypical epithelial proliferation of bile ducts. This case of PISC complicated with atypical biliary glandular changes is described, and the distinction between PISC and carcinoma of the bile duct is discussed. (+info)
Living donor liver transplantation using a right lobe graft in an adult with situs inversus.
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Situs inversus totalis is a rare anatomic variant in which there is a complete mirror image of the thoracic and abdominal viscera. The common association of situs inversus and biliary atresia has led to a variety of modifications of surgical techniques utilizing both living donor and deceased donor liver grafts, with mixed results in the pediatric liver transplant population. The use of a living donor liver graft in an adult with situs inversus has not yet been described. However, living donor liver transplantation (LDLT) has produced excellent results in the adult population, particularly in the cholestatic population, which may be disadvantaged by the model for end-stage liver disease system. This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis. (+info)
Autoimmune cholestatic liver disease in people with coeliac disease: a population-based study of their association.
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BACKGROUND: Population data supporting an association between the autoimmune cholestatic liver diseases, primary biliary cirrhosis and primary sclerosing cholangitis and coeliac disease, is limited and at times contradictory. AIM: To explore the relationship between coeliac disease and both primary biliary cirrhosis and primary sclerosing cholangitis within the General Practice Research Database, a UK-based longitudinal primary care database. METHODS: We identified 4732 people with diagnosed coeliac disease and 23 620 age- and sex-matched controls within the General Practice Research Database. We calculated the prevalence of primary biliary cirrhosis and primary sclerosing cholangitis for both the coeliac disease and control group. RESULTS: There was a higher prevalence of primary biliary cirrhosis in adults with coeliac disease, compared with controls [0.17% vs. 0.05%, odds ratio 3.63 (95% confidence interval: 1.46-9.04)]. Primary sclerosing cholangitis was also more common in the coeliac disease group [0.04% vs. 0%, fishers exact test (P = 0.03)]. CONCLUSIONS: There was a threefold or greater increase in risk of both primary biliary cirrhosis and primary sclerosing cholangitis in people with coeliac disease compared with the general population. The association with primary biliary cirrhosis was weaker than in some reports and it is difficult on the basis of this study to justify screening patients with coeliac disease for either primary biliary cirrhosis or primary sclerosing cholangitis. (+info)
Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic screening for oesophageal varices detection?
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BACKGROUND: Recent guidelines from an AASLD Single Topic Symposium suggest that patients with cirrhosis, including those with primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC), should be screened for oesophageal varices when the platelet count is <140,000/mm3. AIM: To determine the validity of these guidelines in clinical practice in patients with PBC or PSC. METHODS: Retrospective review of individuals undergoing screening upper endoscopy for oesophageal varices at a single centre. Oesophageal varices were reported as being present or absent. RESULTS: A total of 235 patients with chronic liver disease, including 86 patients with PBC (n=79) or PSC (n=7), 104 patients with chronic viral hepatitis, and 45 with non-alcoholic cirrhosis of differing aetiologies, underwent a single screening endoscopy between 1996 and 2001. Oesophageal varices were detected in 26 (30%) of the PBC/PSC group, 38 (37%) of the viral hepatitis group, and 21 (47%) of the "other" group. Applying multiple logistic regression analysis to the data in the group with PBC/PSC, platelets <200,000/mm3 (odds ratio (OR) 5.85 (95% confidence interval (CI) 1.79-19.23)), albumin <40 g/l (OR 6.02 (95% CI 1.78-20.41)), and serum bilirubin >20 micromol/l (OR 3.66 (95% CI 1.07-12.47)) were shown to be independent risk factors for oesophageal varices. Prothrombin time was unhelpful. The values at these cut offs were not useful in predicting oesophageal varices in the other groups. CONCLUSION: We conclude that current guidelines recommended by the AASLD Single Topic symposium are invalid in our cohort of patients with PBC and PSC. Patients with a platelet count <200,000/mm3, an albumin level <40 g/l, and a bilirubin level >20 micromol/l should be screened for oesophageal varices. (+info)
Clinical significance of serum levels of vascular endothelial growth factor and its receptor in biliary disease and carcinoma.
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AIM: To investigate the clinical significance of serum vascular endothelial growth factor (VEGF) and soluble VEGF receptor-1 (VEGFR1/Flt-1) (sVEGFR1) levels in biliary diseases. METHODS: We analyzed the serum levels of these proteins in patients with acute cholangitis (group 1), biliary malignancies (group 2), and primary biliary cirrhosis or primary sclerosing cholangitis (group 3), and in healthy donors (group 4). The influence of inflammation was also analyzed. Serum VEGF levels were expressed as VEGF per platelet (VEGF/PLT, pg/10(6)) in order to exclude the influence of platelet counts. RESULTS: sVEGFR1 levels were significantly higher in groups 1 and 2 than in the control group, but did not correlate with inflammatory markers. VEGF/PLT levels were generally higher in patients with active inflammation than in those with carcinoma. C-reactive protein strongly correlated with the levels of serum VEGF independently of platelet and leukocyte counts, even in cancer patients. In cancer patients, VEGF/PLT and sVEGFR1 levels might be indicators for evaluating the effect of medical treatment or the disease progression. CONCLUSION: Serum VEGF and VEGFR1 might be useful markers for gauging the clinical effect of various treatments on patients. (+info)
The relation between plasma tyrosine concentration and fatigue in primary biliary cirrhosis and primary sclerosing cholangitis.
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BACKGROUND: In primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) fatigue is a major clinical problem. Abnormal amino acid (AA) patterns have been implicated in the development of fatigue in several non-hepatological conditions but for PBC and PSC no data are available. This study aimed to identify abnormalities in AA patterns and to define their relation with fatigue. METHODS: Plasma concentrations of tyrosine, tryptophan, phenylalanine, valine, leucine and isoleucine were determined in plasma of patients with PBC (n = 45), PSC (n = 27), chronic hepatitis C (n = 22) and healthy controls (n = 73). Fatigue and quality of life were quantified using the Fisk fatigue severity scale, a visual analogue scale and the SF-36. RESULTS: Valine, isoleucine, leucine were significantly decreased in PBC and PSC. Tyrosine and phenylalanine were increased (p < 0.0002) and tryptophan decreased (p < 0.0001) in PBC. In PBC, but not in PSC, a significant inverse relation between tyrosine concentrations and fatigue and quality of life was found. Patients without fatigue and with good quality of life had increased tyrosine concentrations compared to fatigued patients. Multivariate analysis indicated that this relation was independent from disease activity or severity or presence of cirrhosis. CONCLUSION: In patients with PBC and PSC, marked abnormalities in plasma AA patterns occur. Normal tyrosine concentrations, compared to increased concentrations, may be associated with fatigue and diminished quality of life. (+info)
Review article: current management of primary sclerosing cholangitis.
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The management of primary sclerosing cholangitis (PSC) is hindered by incomplete understanding of the pathogenesis of the disease and the lack of good prognostic models. Few large randomized controlled trials of drug therapy have been published. Best practice in the management of PSC is currently based therefore on careful interpretation of the available evidence, close observation of individual patients and clinical experience of the disease. Drug therapy is useful for alleviating symptoms. Ursodeoxycholic acid may slow progression of the disease and reduce the frequency of complications. Consensus is emerging on the issues of screening for the malignant complications of PSC and the indications for liver transplantation are becoming broader and encompassing the earliest stages of cholangiocarcinoma. In view of the rarity of the disease in the general population, large international collaborations to study PSC are necessary to provide clearer answers in areas of uncertainty, and these are now beginning to emerge. (+info)