Search results for "primary sclerosing cholangitis"

Mutational Characterization of the Bile Acid Receptor TGR5 in Primary Sclerosing Cholangitis

2010

Background: TGR5, the G protein-coupled bile acid receptor 1 (GPBAR1), has been linked to inflammatory pathways as well as bile homeostasis, and could therefore be involved in primary sclerosing cholangitis (PSC) a chronic inflammatory bile duct disease. We aimed to extensively investigate TGR5 sequence variation in PSC, as well as functionally characterize detected variants.Methodology/Principal Findings: Complete resequencing of TGR5 was performed in 267 PSC patients and 274 healthy controls. Six nonsynonymous mutations were identified in addition to 16 other novel single-nucleotide polymorphisms. To investigate the impact from the nonsynonymous variants on TGR5, we created a receptor mod…

Risk factors of de novo malignancies after liver transplantation: a French national study on 11004 adult patients.

2021

International audience; Background: After liver transplantation (LT),de novo malignancies are one of the leading causes of late mortality. The aim of the present retrospective study was to identify the risk factors of de novo malignancies in a large cohort of LT recipients in France, using Fine and Gray competing risks regression analysis.Methods: The study population consisted in 11004 adults transplanted between 2000 and 2013, who had no history of pre-transplant malignancy, except primary liver tumor. A Cox model adapted to the identification of prognostic factors (competitive risks) was used.Results: From the entire cohort, one (or more)de novo malignancy was reported in 1480 L T recipi…

Guidelines for the diagnosis and management of intrahepatic cholangiocarcinoma

2014

Identification and characterization of autoantibodies against catalase and α-enolase in patients with primary sclerosing cholangitis

1998

SUMMARY Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology. Recent studies have shown that genetic factors and both cellular and humoral immunological abnormalities are important in the pathogenesis of PSC. The most prominent autoantibodies in PSC are anti-neutrophil cytoplasmic antibodies (ANCA). The autoepitopes of ANCA in PSC are not well defined. The aim of this study was to identify corresponding ANCA autoantigens in patients with PSC. A biochemical approach with enrichment and partial purification of soluble neutrophil proteins, detection of autoantibodies by Western blot and partial amino acid sequencing were used. Two new autoantigen/aut…

Autoimmune hepatitis and overlap syndromes

2002

Autoimmune hepatitis (AIH) is an immune-mediated, autodestructive liver disease with hepatocytes as target cells, mostly affecting young women. Primary biliary cirrhosis (PBC) is also regarded as an autoimmune liver disease with bile duct epithelia as the target cells, resulting in a continuous loss of bile ducts. Both diseases may occur simultaneously in their full manifestations in about 10% to 20% of cases, thus constituting an overlap syndrome with PBC directing the course of the disease. AIH may also occur simultaneously with primary sclerosing cholangitis (PSC), with a frequency of between 2% and 8% of patients with PSC. In most cases, AIH precedes manifestation of PSC. In children, t…

Cancer of the Liver and Bile Ducts

2007

Hepatocellular carcinoma (HCC) is the fifth most common neoplasm in the world and the third most common cause of cancer death worldwide. More than 500,000 deaths per year are attributed to HCC, representing 10% of all deaths from cancer. In select areas of Asia and Africa, HCC is the most common cause of death due to cancer. The incidence in Europe and the United States is relatively low but is increasing. In Europe, HCC is now the leading cause of death among patients with cirrhosis. In the United States, epidemiologic studies have demonstrated a doubling of HCC incidence over the past two decades. This increase, which has been attributed to the increasing prevalence of chronic hepatitis C…

Liver transplantation as a primary indication for intrahepatic cholangiocarcinoma: a single-center experience.

2008

Abstract Background Intrahepatic cholangiocarcinoma (ICC) is not a widely accepted indication for orthotopic liver transplantation (OLT). The present study describes our institutional experience with patients who underwent transplantation for ICC as well as those with ICC who underwent transplantation with the incorrect diagnosis of hepatocellular carcinoma (HCC). Patients and Methods Data corresponding to ICC patients were reviewed for the purposes of this study. Patients with hilar cholangiocarcinoma and incidentally found ICC after OLT for benign diseases were excluded from further consideration. Results Among the 10 patients, 6 underwent transplantation before 1996 and 4 after 2001. Tho…

Longterm Survival After Liver Transplantation for Autoimmune Hepatitis : Results From the European Liver Transplant Registry

2020

The aim of this study was to analyze longterm patient and graft survival after liver transplantation for autoimmune hepatitis (AIH-LT) from the prospective multicenter European Liver Transplant Registry. Patient and liver graft survival between 1998 and 2017 were analyzed. Patients after AIH-LT (n = 2515) were compared with patients receiving LT for primary biliary cholangitis (PBC-LT; n = 3733), primary sclerosing cholangitis (PSC-LT; n = 5155), and alcohol-related cirrhosis (AC-LT; n = 19,567). After AIH-LT, patient survival was 79.4%, 70.8%, and 60.3% and graft survival was 73.2%, 63.4%, and 50.9% after 5, 10, and 15 years of follow-up. Overall patient survival was similar to patients af…

Diagnostik der primär sklerosierenden Cholangitis: prospektiver Vergleich von MR-Cholangiographie mit endoskopisch retrograder Cholangiographie

1998

PURPOSE To assess the accuracy of MR cholangiography (MRC) in the diagnosis of primary sclerosing cholangitis (PSC) in comparison to endoscopic retrograde cholangiography (ERC). METHOD 20 patients with PSC were examined by ERC and MRC (1.0 T, HASTE sequence). Visualization and pathologic changes of the extra- and intrahepatic bile ducts were evaluated with both methods. RESULTS Mural irregularities of the common bile duct were seen with MRC in 6/7 cases, stenoses and dilatation of the common bile duct were detected correctly in all patients. Diffuse, multifocal strictures of the intrahepatic bile ducts were the most common intrahepatic findings and correctly diagnosed in almost all patients…

Clinical Presentation of Crohn’s Disease

2015

Crohn’s disease is an idiopathic chronic inflammatory disease of the gut, which may involve the entire gastrointestinal tract from the mouth to the perianal area, though preferring in most cases the distal small bowel and the proximal large bowel. Its heterogeneous nature is reflected in a number of different phenotypes. Approximately 80 % of patients have small bowel involvement, usually in the distal ileum, with one-third of patients having exclusively ileitis. Approximately 50 % of patients have ileocolitis which refers to involvement of both the ileum and colon. From 20 to 25 % of patients have disease confined to the colon. Involvement of the esophagus, stomach, or duodenum is rare and…