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Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis is a rare, chronic liver disease characterized by inflammation and scarring of the bile ducts, both inside and outside the liver.
This condition can lead to the buildup of bile, liver damage, and potentially life-threatening complications.
Researchers can leverage PubCompare.ai's powerful AI-driven platform to effortlessly locate the best research protocols and products related to Primary Sclerosing Cholangitis, optimizing their research joruney and uncovering valuable insights to advance understanding and treatment of this complex condition.

Most cited protocols related to «Primary Sclerosing Cholangitis»

In brief, enrollees in the prospective study were persons receiving single or multiple drugs, herbals, or other over-the-counter products identified to have biochemically defined liver dysfunction, provided that they could be evaluated within 6 months of onset of the liver disease.14 (link) Biochemical criteria for enrollment included (1) two consecutive serum alanine aminotransferase (ALT) or aspartate aminotransferase (AST) values > 5 times the upper limit of normal (ULN) or > 5 times the baseline abnormal value, (2) two consecutive serum alkaline phosphatase (AP) values greater than twice the ULN or twice the baseline abnormal value, or (3) an otherwise unexplained total serum bilirubin value > 2.5 mg/dL or an international normalized ratio (INR) > 1.5 on two consecutive occasions. Symptoms or signs of liver injury were not required. Exclusion criteria were liver injury due to acetaminophen, preexisting autoimmune hepatitis or sclerosing cholangitis, and previous receipt of a bone marrow or liver transplant. Persons were not excluded for preexisting chronic hepatitis B or C or human immunodeficiency virus infection, provided that baseline laboratory test results were available.
Publication 2010
Acetaminophen Alanine Transaminase Alkaline Phosphatase Autoimmune Chronic Hepatitis Bilirubin Bone Marrow Drugs, Non-Prescription Hepatitis B, Chronic HIV Infections Injuries International Normalized Ratio Liver Liver Diseases Liver Transplantations Pharmaceutical Preparations Primary Sclerosing Cholangitis Serum Transaminase, Serum Glutamic-Oxaloacetic
The OPTN database of the United Network for Organ Sharing (UNOS) was used to identify patients registered on the waiting list on or before April 24, 2006, during 2 periods, pre-MELD (January 1, 1996–December 31, 2000) and post-MELD (February 28, 2002–March 31, 2006). The study population comprised all non-Hispanic black and non-Hispanic white patients aged 18 years or older who were liver transplant waiting list registrants during those periods. Race is identified by patients when registering on the UNOS waiting list. Hispanics were not included as the UNOS database has multiple variables that identify race and ethnicity with often discordant results.
Patients listed for retransplantation or multiorgan transplantation were excluded. Given the different criteria for organ allocation, patients were excluded if they were listed as status 1, defined as fulminant liver failure with a life expectancy without liver transplantation of less than 7 days. We also excluded patients listed as temporarily inactive because they could not be properly assessed for receipt of liver transplantation. The UNOS variables collected included listing date, age, sex, blood type, listing diagnoses, race, education level, insurance payer, UNOS region, calculated MELD score at listing and removal, waiting time, reason for removal from the waiting list, and comorbid illnesses. Waiting time was determined using the first date each patient was placed on the waiting list and the date of removal from the list.
Education level was grouped into 3 categories comprising no education or grade school education, high school and attended college without degree, and college degree or higher. The UNOS regions were grouped into 4 categories according to region of the country (northeast = UNOS regions 1, 2, and 9; southeast = UNOS regions 3, 4, and 11; midwest = UNOS regions 7, 8, and 10; and west = UNOS regions 5 and 6). Listing diagnoses were grouped into 11 common diagnostic categories including cryptogenic cirrhosis, hepatitis C virus, hepatitis B virus, Laennec cirrhosis, nonalcoholic steatohepatitis, autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, alpha-1 antitrypsin deficiency, hemochromatosis, and HCC. All other diagnoses were combined into a category designated “other.”
Patients with reasons for removal from the waiting list, such as emergency transplant, died during transplant, and transplanted at another center, were combined into a category called “transplanted.” Similarly, patients removed from the waiting list with reasons such as medically unsuitable or too sick for liver transplantation were combined into a category called “too sick for liver transplantation,” and patients whose reasons for removal from the waiting list were identified as refused transplant, transferred to another center, other, condition improved, living donor, and removed in error were combined into a category called “other.” Patients who died before liver transplantation were kept as 1 category and called “died.”
Publication 2008
alpha 1-Antitrypsin Deficiency Autoimmune Chronic Hepatitis B virus, Hepatitis Cirrhosis, Cryptogenic Diagnosis Emergencies Ethnicity Hemochromatosis Hepatitis C virus Hispanics Liver Cirrhosis Liver Failure, Acute Liver Transplantations Living Donors Nonalcoholic Steatohepatitis Patients Primary Biliary Cholangitis Primary Sclerosing Cholangitis Transplantation
The study protocol was approved by the local ethics committee (ethics committee of University Hospital Aachen, RWTH Aachen), and written informed consent was obtained from each patient. The study was conducted according to the principles expressed in the Declaration of Helsinki. Inclusion criteria were either any CLD with a predisposition to liver fibrosis or an already established liver fibrosis/cirrhosis of any origin. Established cirrhosis (in contrast to non-cirrhotic CLD) was defined, if imaging (ultrasound, CT or MRI scan), biopsy or laparoscopy indicated liver cirrhosis or if cirrhosis-related complications were present. Patients with established liver cirrhosis were staged according to Child-Pugh's criteria [32] (link). Patients with acute liver failure or acute hepatitis B or C were not included. Exclusion criteria were conditions known to directly affect monocyte subset distributions in humans, specifically ongoing bacterial infections (procalcitonin concentration above normal value [<0.5 µg/L]), HIV-infection, systemic steroid medication (prednisolone >7.5 mg/d or equivalent doses) and malignant tumor(s) except hepatocellular or cholangiocellular carcinoma. Furthermore, patients were excluded in case of systemic inflammatory response syndrome (SIRS) or sepsis criteria [33] (link). The etiologies of liver diseases comprised viral hepatitis (n = 89, 39.4%; HBV n = 38, HCV n = 51), biliary or autoimmune disease (n = 27, 11.9%; autoimmune hepatitis n = 10, primary biliary cirrhosis n = 8, primary sclerosing cholangitis n = 9), alcoholic liver disease (n = 65, 28.7%) and other liver diseases (n = 45, 20%, e.g. non-alcoholic steatohepatitis n = 7, hemochromatosis n = 4, cryptogenic n = 23). Grading and staging of liver samples (biopsies and explants) were performed according to Desmet-Scheuer score by one experienced pathologist, who was fully blinded to any experimental data [34] (link).
As a control group, 181 healthy volunteers were recruited from the local blood transfusion institute that had normal aminotransferase activities, no history of liver disease or alcohol abuse and tested negative for HBV, HCV and HIV infections.
Publication 2010
Abuse, Alcohol Alcoholic Liver Diseases Autoimmune Chronic Hepatitis Autoimmune Diseases Bacterial Infections Bile Biopsy Blood Transfusion Child Cholangiocarcinoma Ethics Committees, Clinical Fibrosis Fibrosis, Liver Healthy Volunteers Hemochromatosis Hepatitis B Hepatitis Viruses HIV Infections Homo sapiens Laparoscopy Liver Liver Cirrhosis Liver Diseases Liver Failure, Acute Malignant Neoplasms Monocytes MRI Scans Nonalcoholic Steatohepatitis Pathologists Patients Pharmaceutical Preparations Prednisolone Primary Biliary Cholangitis Primary Sclerosing Cholangitis Procalcitonin Regional Ethics Committees Septicemia Steroids Susceptibility, Disease Systemic Inflammatory Response Syndrome Transaminases Ultrasonics
A description of the mouse and human tissues and experimental procedures is available in the Supporting Information. The present analysis included transcriptome data from seven mouse models (one with chronic and six with acute liver damage) consisting of 227 mice (Table 1; Supporting Fig. S1A) and five studies of human CLD with a total of 372 patients (Table 1; Supporting Fig. S1B). The analyzed data sets were either generated in‐house (all mouse models except tunicamycin) or downloaded from public sources (acute tunicamycin model and all human data sets). Additionally, we analyzed nine published sets of differentially expressed genes of CLD mouse models, for which the corresponding raw data were not available.(3) Biopsies from patients with primary sclerosing cholangitis (PSC) and alcohol‐associated liver disease were used for validation by immunostaining (Supporting Table S1).
Publication 2021
Alcoholic Liver Diseases Biopsy Homo sapiens Liver Mice, Laboratory Patients Primary Sclerosing Cholangitis Tissues Transcriptome Tunicamycin

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Publication 2011
Autoimmune Chronic Hepatitis Bile Acids Biliary Atresia Child Cholestasis, progressive familial intrahepatic 2 Cystic Fibrosis Glycogen Storage Disease Hepatic Fibrosis, Congenital Hepatitis A Hepatitis Viruses Homo sapiens Immunosuppression Liver Liver Diseases Liver Failure, Acute Metabolic Diseases Methylmalonic acidemia Nonalcoholic Steatohepatitis Ornithine Carbamoyltransferase Deficiency Patients Primary Sclerosing Cholangitis SERPINA1 protein, human Woman

Most recents protocols related to «Primary Sclerosing Cholangitis»


This prospective observational study to determine occupational radiation doses was performed at the Helsinki University Hospital Endoscopy department between March 2021 and July 2021. The COVID-19 pandemic did not affect the number or type of performed procedures. Altogether 604 consecutive fluoroscopy-guided procedures to patients were included in the study. From these interventions, 560 were ERCPs and 44 were other gastrointestinal endoscopy procedures, such as duodenal stentings or dilatations of anastomotic strictures. Personal dose equivalents H
p(10), H
p(0.07), and H
p(3) for four gastrointestinal surgeons (S1-S4) and four gastroenterologists (G1-G4) and for assisting nurses (N_Zee and N_Cios) were measured using thermoluminescent dosimeters (TLD) and direct-ion storage dosimeters (DIS). Details of dosimetry practices and dose uncertainty estimation are provided as supplementary material. In the endoscopy department, ERCPs for diagnosis and follow up of primary sclerosing cholangitis (PSC) and dilatations and stentings for these patients are performed by gastroenterologists; surgeons perform all other ERCP procedures. Distributions of the performed and assisted procedures by endoscopist and assisting nurse are given in Table 1 s (supplementary materials). The study was approved by the Institutional Review Board and no patient informed consent was required.
Publication 2023
COVID 19 Cranioosteoarthropathy Diagnosis Dilatation Duodenum Endoscopic Retrograde Cholangiopancreatography Endoscopy, Gastrointestinal Ethics Committees, Research Fluoroscopy Gastroenterologist Nurses Patients Primary Sclerosing Cholangitis Radiometry Radiotherapy Stenosis Stents Surgeons Surgical Anastomoses Surgical Procedures, Endoscopic Gastrointestinal
IBD diagnosis was made by an expert gastroenterologist by synthesizing various findings, such as conventional clinical, endoscopic, radiologic, and histopathologic results. CD was defined as disease location (L1, ileal; L2, colonic; L3, ileocolonic; L4, and upper gastrointestinal tract) and disease behavior (B1, nonstricturing, nonpenetrating; B2, stricturing; B3, and penetrating) according to the Montreal classification. UC is classified into proctitis (E1), left-sided colitis (E2), and extended colitis, including pancolitis (E3), and according to the degree of disease extent.[13 ]EIMs were diagnosed and treated by a specialist according to their subtypes. Depending on the organ involved, EIMs were defined as articular (peripheral arthritis, ankylosing spondylitis, and sacroiliitis), cutaneous (erythema nodosum, pyoderma gangrenosum, and others), ocular (uveitis and episcleritis), or hepatobiliary (primary sclerosing cholangitis). Patients with 2 or more EIMs were classified according to the number of EIMs.
Publication 2023
Ankylosing Spondylitis Arthritis Colitis Colon Endoscopy Episcleritis Erythema Nodosum Eye Gastroenterologist Ileum Joints Patients Primary Sclerosing Cholangitis Proctitis Pyoderma Gangrenosum Sacroiliitis Skin Upper Gastrointestinal Tract Uveitis
The present study was a retrospective hospitalization-based cross-sectional study, which was approved by the Ethics Committees of Fifth Medical Center of Chinese PLA General Hospital (No. 2019024D). Written informed consent for liver biopsy was obtained from all patients, whereas patient consent for data collection was waived due to the study design.
The inclusion criteria included the following: 1) admitted from June 2005 to September 2022; 2) age ≥60 years old; 3) met the DILI definition (see definition section); 4) Roussel Uclaf Causality Assessment Method (RUCAM) score >6 points; and 5) the diagnosis of DILI was confirmed by liver biopsy. The exclusion criteria were as follows: 1) those with any other definite etiologies of liver disease (e.g., primary biliary cholangitis, primary sclerosing cholangitis, viral hepatitis, alcoholic or non-alcoholic liver disease, Gilbert syndrome, etc.) according to their relevant guidelines (Lindor et al., 2015 (link); EASL–EASD–EASO Clinical Practice, European Association for the Study of Diabetes EASD, European Association for the Study of Obesity EASO, 2016 (link); Hirschfield et al., 2017 (link); Singal et al., 2018 (link); Wagner et al., 2018 (link); Te and Doucette, 2019 (link)); 2) those with severe systemic diseases affecting the liver (heart attack, stroke, kidney, or HIV infection); and 3) those with incomplete important data.
Publication 2023
Alcoholic Liver Diseases Alcoholics Biopsy Cerebrovascular Accident Chinese Diabetes Mellitus Diagnosis Ethics Committees, Clinical Europeans Gilbert Disease Hepatitis Viruses HIV Infections Hospitalization Kidney Liver Liver Diseases Myocardial Infarction Obesity Patients Primary Biliary Cholangitis Primary Sclerosing Cholangitis
We recorded patient’s age at study enrollment, sex, and a constructed variable from self-reported race and ethnicity in the survey (non-Hispanic white, non-Hispanic Black, Hispanic, and other groups). Etiology of cirrhosis was active HCV if the patient had a positive HCV RNA test, and cured HCV if there was documentation of treatment and subsequent sustained virological response (SVR) at the time of enrollment [20 (link)]; HBV was based on a positive HBsAg [21 (link), 22 (link)]; and alcohol-related cirrhosis based on a combination of clinician-recorded diagnosis of alcoholic liver disease and patients’ self-report of former heavy (8 or more alcoholic beverages per week for women or 15 or more alcoholic beverages per week for men) or any current use of alcohol. We used a validated survey to ascertain alcohol use that classified alcohol use status as lifetime abstention (never), former light to moderate use, former heavy use, current light to moderate use, and current heavy use. We used clinician-recorded diagnosis to define other etiologies, including autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, hereditary hemochromatosis, and Wilson’s disease. The diagnosis of NAFLD required documentation of hepatic steatosis on liver histology or imaging and the absence of HCV (active/untreated or resolved HCV), HBV, alcoholic liver disease, or other clinician documented etiologies listed above. Most (>90%) patients classified as NAFLD also had a clinician-recorded diagnosis of NAFLD in the EMR.
We defined diabetes, hypertension, and dyslipidemia based on patients’ medical history (survey or EMR) or self-reported treatment with diabetes medications (oral hypoglycemic medications or insulin), antihypertensives, or treatments for dyslipidemia (bile acid–binding resins, β-hydroxy β-methylglutaryl-CoA reductase inhibitors, nicotinic acid and fibric acid derivatives) at any time before enrollment. We defined smoking status as never, past, and current smoker based on self-report using the baseline survey. We used height and weight values at enrollment to calculate body mass index (BMI; kg/m2).
Publication 2023
Alcoholic Beverages Alcoholic Liver Diseases Antihypertensive Agents Autoimmune Chronic Hepatitis Bile Acids Diabetes Mellitus Diagnosis Dyslipidemias Ethnicity Fatty Liver Fibric Acids Hemochromatoses, Familial Hepatitis B Surface Antigens Hepatolenticular Degeneration High Blood Pressures Hispanics Hypoglycemic Agents Index, Body Mass inhibitors Insulin Light Liver Liver Cirrhosis Liver Cirrhosis, Alcoholic Niacin Non-alcoholic Fatty Liver Disease Oxidoreductase Patients Pharmacotherapy Primary Biliary Cholangitis Primary Sclerosing Cholangitis Resins, Plant Woman
HBV-seropositive patients who received clinical observation at Kyoto University Hospital and Osaka Red Cross Hospital between April 2001 and March 2020 were enrolled in this study. The inclusion criteria were: consistently seropositive for HBsAg serum HBV-DNA titer higher than 4.23 LogIU/mL (105 copies/mL) for at least 2 time-points; free from antiviral medications or interferon treatment. The exclusion criteria were: under 20 years old; coinfection with HCV, HIV, or human T-lymphotropic virus; uncontrolled malignancies; liver comorbidities such as alcohol-associated liver disease, nonalcoholic steatohepatitis, autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, immunoglobulin G4-related sclerosing cholangitis, or Wilson disease. Two to 5 serum samples were sequentially collected during the observation period. All samples were surpluses of sera drawn for clinical purposes. The collected samples were stored at −80°C.
This study conformed to the provisions of the Declaration of Helsinki. The study protocol (R2594) was approved by the ethics committee of Kyoto University and the clinical samples were obtained with written informed consent or based on an opt-out method of consent from all participants.
Publication 2023
Alcoholic Liver Diseases Antiviral Agents Autoimmune Chronic Hepatitis Coinfection Ethics Committees Hepatitis B Surface Antigens Hepatolenticular Degeneration IgG4 Interferons Malignant Neoplasms ML 23 Nonalcoholic Steatohepatitis Patients Primary Biliary Cholangitis Primary Sclerosing Cholangitis Serum Specimen Collection T-Cell Leukemia Viruses, Human

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More about "Primary Sclerosing Cholangitis"

Primary Sclerosing Cholangitis (PSC) is a rare, chronic liver disease characterized by inflammation and scarring of the bile ducts, both inside and outside the liver.
This condition, also known as sclerosing cholangitis, can lead to the buildup of bile, liver damage, and potentially life-threatening complications.
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