Clinical Application of Annual Liver Multiscan and MRCP+ in Primary Sclerosing Cholangitis
Primary Sclerosing CholangitisPrimary sclerosing cholangitis (PSC) is a chronic progressive biliary disease that affects approximately 1200 patients in the Netherlands and around 80,000 in the Western world. It is often accompanied by ulcerative colitis (UC) or Crohn's disease affecting the large bowel. The cause of PSC is unknown, there is no medical therapy available that has proven to halt disease progression and the median time until death or liver transplantation is 13-21 years. Diagnosis is made by magnetic resonance cholangiography (MRC), or in the case of so called small duct disease by liver biopsy. Due to the heterogeneous disease course and the relatively low clinical event rate of 5% per year it is difficult to predict prognosis of individual patients or to recommend any surveillance strategy for malignancies. Also, the lack of surrogate endpoints impedes performing clinical research. Recently, two new post-processing tools have been developed to characterize and quantify abnormalities in the biliary tree as well as excretory function captured by MRC. These tools called MRCP+ (quantitative magnetic resonance cholangiopancreatography +) and LiverMultiscan (LMS) hold the prospect of adequately depicting and quantifying lesions of the biliary tree as well as capturing functional derailment. However, several features must be tested before the utility of this tools in clinical patient care can be concluded. Therefore, the aim of this study is to investigate the utility of these novel techniques in monitoring disease activity by performing consecutive annual MRI's.
Multicenter Observational Study of PSC and IgG4-SC in China
Primary Sclerosing CholangitisIgG4-related Sclerosing CholangitisThe investigators aimed to collect demographic features and clinical outcomes in patients diagnosed with PSC and IgG4-SC by utilizing participants database from multiple medical centers across Mainland China. Cross-sectional studies will focus on characterizing clinical presentations and validating diagnostic and prognostic models on Chinese PSC and IgG4-SC patients.
Secondary Sclerosis Cholangitis Prospective
Secondary Sclerosis Cholangitis in Critically Ill PatientsSC-CIP is increasing in patients after critical illness. Pathogenesis is still largely unclear. Gut microbiome composition, gut permeability, bacterial translocation, inflammation and/or genetic variants contribute to the pathogenesis The aim of this project is to study gut microbiome composition, gut permeability, bacterial translocation, inflammation, bile acid composition and genetic polymorphisms by conducting a prospective cohort study in patients with a high risk to develop SC-CIP.
Biobank for Cholestatic Liver Diseases.
Primary Sclerosing CholangitisPrimary Biliary Cirrhosis2 moreThis study is a biobank of specimens and clinical data for use in current and future research to better understand the cholestatic liver diseases primary biliary cirrhosis/cholangitis (PBC) and primary sclerosing cholangitis (PSC).
Characterization of Biliary Cell-derived Organoids From Bile of PSC and Non-PSC Patients
Primary Sclerosing CholangitisPSC1 moreThe purpose of this research is to create a collection of bile, bile duct brushings and medical information from people with Primary Sclerosing Cholangitis (PSC) and controls to learn more about changes that occur in the liver.
Performance of Scoring Systems in Chinese Patients With Primary Biliary Cholangitis (PBC) on Ursodeoxycholic...
Primary Biliary Cholangitis (PBC)Several risk assessment scoring systems have been proposed to assess the therapeutic response and predict long term prognosis in ursodeoxycholic acid (UDCA)-treated primary biliary cholangitis (PBC) patients, in order to risk stratify PBC patients and guide their management. However there scoring systems have not been fully validated in Chinese population. This study is going to compare the prognostic ability of these criteria, validate the overseas scoring systems, develop and validate a new scoring system in a cohort of Chinese PBC patients.
Swiss Primary Biliary Cholangitis Cohort Study
Liver CirrhosisBiliaryResearch project in which biological material is sampled and health-related medical data is collected. In addition, already existing health-related medical data are used for further research. Coded data are used.
Swiss Primary Sclerosing Cholangitis Cohort Study
CholangitisSclerosingResearch project in which biological material is sampled and health-related personal data is further used and collected. Coded data are used.
MRI Based Biomarkers in Pediatric Autoimmune Liver Disease
Autoimmune Liver DiseasePrimary Sclerosing Cholangitis1 moreAutoimmune liver diseases (AILD), which include Primary Sclerosing Cholangitis (PSC) and Autoimmune Hepatitis (AIH) are a common etiological factors for chronic liver disease among adolescents. In all these conditions, autoimmune lymphocyte responses are thought to orchestrate inflammatory injury against hepatocytes (primarily in AIH) or cholangiocytes (in PSC). In this proposal we aim to evaluate the Magnetic Resonance Imaging (MRI) modalities; MR cholangiopancreatography (MRCP) and MR elastography (MREL), as non-invasive biomarkers to assess two primary pathophysiological processes of AILD: bile duct damage and liver fibrosis. In this cross-sectional study MRI based findings of bile duct injury and liver fibrosis will be correlated with both liver histology and circulating biomarkers of these disease processes.
Predictive Models of Hepatic Decompensation and Survival Outcomes in Pediatric Patients With Cirrhosis...
CirrhosisBiliary Atresia5 moreThe aim of this study was to developed and validated models to predict hepatic decompensation and survivals in pediatric patients with cirrhosis and compared these models with currently available models.