Incidence of hepatobiliary malignancies in primary sclerosing cholangitis: systematic review and meta-analysis.

Background And Aims: Primary sclerosing cholangitis (PSC) is a known risk factor for hepatobiliary malignancies. We conducted a systematic review and meta-analysis of published studies to determine the incidence and risk factors for hepatobiliary malignancies in people with PSC.

Methods: Pubmed and Embase databases were searched from inception to April 10, 2024 for cohort studies reporting data on the incidence of cholangiocarcinoma (CCA), hepatocellular carcinoma (HCC), or gallbladder cancer (GBC) in PSC.

Determining the time to cholangiocarcinoma in pediatric-onset PSC-IBD.

Primary sclerosing cholangitis (PSC) is a risk factor for cholangiocarcinoma. When a child is diagnosed with both PSC and inflammatory bowel disease (IBD), evidence-based information on counseling families and risk management of developing cholangiocarcinoma is limited. In this case series (PubMed/collaborators), we included patients with PSC-IBD who developed cholangiocarcinoma and contacted authors to determine an event curve specifying the time between the second diagnosis (IBD or PSC) and a diagnosis of cholangiocarcinoma.

PPAR agonists for the treatment of cholestatic liver diseases: Over a decade of clinical progress.

Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are characterized by the destruction of the small bile ducts and the formation of multifocal biliary strictures, respectively, impairing bile flow. This leads to the hepatic accumulation of bile acids, causing liver injury and the risk of progression to cirrhosis and liver failure. First-line therapy for PBC is ursodeoxycholic acid, although up to 40% of treated individuals are incomplete responders, and there is no effective therapy for PSC, highlighting the need for better therapeutic options in these diseases.

Enterococcus avium Infection After Liver Transplantation in Vietnam: A Case Report.

Background: The infectious complications are the most common and can be life-threatening to liver transplant recipients, in particular, within the first month after transplantation. Early diagnosis of these severe complications and accurate detection of causative etiologies are crucial for the choice of therapeutic strategies and management of liver transplants.

Case Report: We present a case report of a patient with a history of primary sclerosing cholangitis who underwent a liver transplantation (LT) from a living donor.

Intestinal histopathology in pediatric PSC-IBD: Characterization of phenotype and assessment of the Nancy Index.

Background: We aimed to characterize the histologic gut phenotype of pediatric primary sclerosing cholangitis (PSC)-associated inflammatory bowel disease (IBD) against non-PSC colitis, and to assess Nancy Index (NI) performance in pediatric PSC-IBD.

Methods: Single-center retrospective cohort study including children diagnosed with PSC-IBD or non-PSC colitis (ulcerative colitis [UC] or IBD-unclassified) from 2000 to 2018, with diagnostic intestinal biopsies. Biopsies were re-reviewed by two independent pathologists who assessed microscopic disease distribution, NI scores, and specific histological features in the right and left colons, overall and stratified by endoscopic severity (moderate-severe vs.

Natural History and Predictors of Clinical Outcomes in Autoimmune Liver Diseases: A Multicenter Study.

Background And Aim: Prognosis in autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) has historically been poor. This multicenter retrospective cohort study investigated the natural history and assessed the predictors of outcomes in patients with AIH, PBC, and PSC.

Methods: AIH, PBC, and PSC patients were identified from the state-wide Hepascore and Clinical Outcome cohort.

Cytoskeleton Remodeling-Related Proteins Represent a Specific Salivary Signature in PSC Patients.

Primary sclerosing cholangitis (PSC) and Primary biliary cholangitis (PBC) are chronic inflammatory biliary diseases characterized by progressive damage of the bile ducts, resulting in hepatobiliary fibrosis and cirrhosis. Currently, specific biomarkers that allow to distinguish between PSC and PBC do not exist. In this study, we examined the salivary proteome by carrying out a comprehensive and non-invasive screening aimed at highlighting possible quali-quantitative protein deregulations that could be the starting point for the identification of effective biomarkers in future.

Molecular Mechanisms of Fibrosis in Cholestatic Liver Diseases and Regenerative Medicine-Based Therapies.

The aim of this review is to explore the potential of new regenerative medicine approaches in the treatment of cholestatic liver fibrosis. Cholestatic liver diseases, such as primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and biliary atresia (BA), due to the accumulation of bile, often progress to liver fibrosis, cirrhosis, and liver failure. When the disease becomes severe enough to require liver transplantation.

Clostridium Scindens Protects Against Vancomycin-Induced Cholestasis and Liver Fibrosis by Activating Intestinal FXR-FGF15/19 Signaling.

Primary sclerosing cholangitis (PSC) is characterized by abnormal bile acid metabolites and altered gut microbiota, with no effective treatments available. Vancomycin, a glycopeptide antibiotic, has emerged as a promising candidate. However, the mechanism by which vancomycin impacts the progression of PSC remains unknown.

Perihilar Cholangiocarcinoma Originating in Peribiliary Glands: Insights from a Case without Precancerous Lesions.

BACKGROUND Recent studies have shown that peribiliary glands may be the potential cell origin of cholangiocarcinoma, and that precancerous lesions such as biliary intraepithelial neoplasms and intraductal papillary neoplasms of the bile duct may arise from these peribiliary glands. However, whether and how these precancerous lesions progress to cholangiocarcinoma is controversial. CASE REPORT Herein, an autopsy case of perihilar cholangiocarcinoma, exclusively periductal-infiltrating, is reported.

Outcome of Liver Retransplantation in Patients With Primary Sclerosing Cholangitis.

Background And Aims: Primary sclerosing cholangitis (PSC) is among the most common indications for liver transplantation in the Nordic countries and with an increasing trend in Europe and North America. Due to post-transplant complications and high prevalence of disease recurrence this group is at risk of requiring retransplantation (re-LTX). Results from re-LTX for PSC are not extensively studied and there is a lack of knowledge regarding prognosis after re-LTX in this population.

Open label vancomycin in primary sclerosing cholangitis-inflammatory bowel disease: improved colonic disease activity and associations with changes in host-microbiome-metabolomic signatures.

Background: We conducted a single-arm interventional study, to explore mucosal changes associated with clinical remission under oral vancomycin (OV) treatment, in primary sclerosing cholangitis associated inflammatory bowel disease (PSC-IBD); NCT05376228.

Method: Fifteen patients with PSC and active colitis (median faecal calprotectin 459µg/g; median total Mayo score 5) were treated with OV (125mg QID) for 4 weeks and followed-up for a further 4 weeks of treatment withdrawal (8 weeks, end-of-study). Colonic biopsies were obtained at baseline and week 4.

Quantitative fibrosis identifies biliary tract involvement and is associated with outcomes in pediatric autoimmune liver disease.

Background: Children with autoimmune liver disease (AILD) may develop fibrosis-related complications necessitating a liver transplant. We hypothesize that tissue-based analysis of liver fibrosis by second harmonic generation (SHG) microscopy with artificial intelligence analysis can yield prognostic biomarkers in AILD.

Methods: Patients from single-center studies with unstained slides from clinically obtained liver biopsies at AILD diagnosis were identified.

Acute Viral Hepatitis E Presenting With Bell's Palsy and Acute Pancreatitis: A Case Report.

Hepatitis E is a hepatotropic virus and the most common cause of acute viral hepatitis among adults in India. It has four genotypes, and genotype 1 is mostly associated with sporadic cases. It typically causes self-limiting acute hepatitis following a prodromal course.

Serum IgG4-negative and IgG4-positive type 1 autoimmune pancreatitis present with different clinicopathological features: An analysis of a nationwide survey in Japan.

Background/objective: Elevated serum IgG4 (sIgG4) is a useful diagnostic marker of type 1 autoimmune pancreatitis (AIP). This study aimed to clarify the clinicopathological characteristics of the type 1 AIP patients without elevated sIgG4 levels.

Methods: We analyzed the clinical data of patients registered in a nationwide epidemiological survey in Japan.

Secondary sclerosing cholangitis due to drug-induced liver injury: a retrospective cohort study.

Background: The main objective of our study was to assess the frequency of drug-induced liver injury (DILI) patients with coexisting secondary sclerosing cholangitis (SSC) within our center and then analyze clinical features of these patients. SSC has received limited attention in the context of DILI. These changes can be observed on magnetic resonance cholangiopancreatography (MRCP).

Secondary Sclerosing Cholangitis due to Drugs With a Special Emphasis on Checkpoint Inhibitors.

Background: Secondary sclerosing cholangitis (SSC), is one of the phenotypes of DILI first described in the 1980s. Check point inhibitors (CPIs) are currently the most frequent cause of SCC.

Aims: To describe the epidemiology, clinical and biochemical features at presentation, differential diagnoses, pathophysiology, imaging, histological characteristics and management associated with SSC.