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Primär sklerosierende Cholangitis: Latest results from PubMed
Safety and sustained efficacy of the farnesoid X receptor (FXR) agonist cilofexor over a 96-week open-label extension in patients with PSC
CONCLUSIONS: In this 96-week OLE of a phase 2 study of PSC, cilofexor was safe and improved liver biochemistry and biomarkers of cholestasis and cellular injury.
07.08.2022 12:00
Liver organoids: From fabrication to application in liver diseases
As the largest internal organ, the liver is the key hub for many physiological processes. Previous research on the liver has been mainly conducted on animal models and cell lines, in which not only there are deficiencies in species variability and retention of heritable material, but it is also difficult for primary hepatocytes to maintain their metabolic functions after in vitro expansion. Because of the increased burden of liver disease worldwide, there is a growing demand for 3D in vitro...
04.08.2022 12:00
ERCP-Related Adverse Events in Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis
CONCLUSIONS: In the context of limited comparative data and heterogeneity, PSC patients undergoing ERCP have higher odds of cholangitis despite the majority receiving antibiotics. Additionally, accidental wire passage and biliary sphincterotomy increased the odds of PEP. Future studies on ERCP-related risks and preventive strategies are needed.
01.08.2022 12:00
Genetic alterations during the neoplastic cascade towards cholangiocarcinoma in primary sclerosing cholangitis
Carcinogenesis of primary sclerosing cholangitis (PSC)-associated cholangiocarcinoma (CCA) is largely unexplored. Improved understanding of the molecular events involved may guide development of novel avenues for rational clinical management. We aimed to assess the genetic alterations during progression of the neoplastic cascade from biliary dysplasia towards CCA in PSC. Forty-four resection specimens or biopsies of PSC patients with biliary dysplasia (n=2) and/or CCA (n=42) were included. DNA...
28.07.2022 12:00
Do Patients with Autoimmune Conditions Have Less Access to Liver Transplantation despite Superior Outcomes?
Orthotopic liver transplantation (OLT) is a lifesaving therapy for patients with irreversible liver damage caused by autoimmune liver diseases (AutoD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC). Currently, it is unclear how access to transplantation differs among patients with various etiologies of liver disease. Our aim is to evaluate the likelihood of transplant and the long-term patient and graft survival after OLT for...
27.07.2022 12:00
Recent Insights into Pediatric Primary Sclerosing Cholangitis
This article reviews recent literature on the pathogenesis, presentation, diagnosis, comorbidities, natural history, and management of pediatric primary sclerosing cholangitis (PSC). The authors shed light on the role of genetic and environmental factors in PSC, although recognize the limitations in the understanding of PSC pathogenesis. They reflect on presenting disease phenotypes, including the association with inflammatory bowel disease and frequent histologic presence of autoimmune...
22.07.2022 12:00
Prolonged administration of melatonin ameliorates liver phenotypes in cholestatic murine model
BACKGROUND AND AIMS: Primary sclerosing cholangitis (PSC) is characterized by biliary senescence and hepatic fibrosis. Melatonin exerts its effects by interacting with MT1/MT2 melatonin receptors. Short-term (1 wk) melatonin treatment reduces ductular reaction (DR) and liver fibrosis in (i) bile duct ligated rats by downregulation of MT1 and clock genes; and (ii) Mdr2^(-/-) mice by decreased miR200b-dependent angiogenesis. We aimed to evaluate the long-term effects of melatonin on liver...
21.07.2022 12:00
Quality of life (QoL) for people with primary sclerosing cholangitis (PSC): a pragmatic strategy for identifying relevant QoL issues for rare disease
CONCLUSION: We identified a range of QoL issues relevant to PwPSC, with a good breadth of coverage, although lacking an in-depth understanding of the PSC experience. Our strategy effectively identified relevant QoL issues for people living with this rare condition, for which there is no consensus on stratifying for its severity. This strategy should however be considered specific to such circumstances, not a general recommendation for an alternative approach.
15.07.2022 12:00
Fenofibrate Downregulates NF-κB Signaling to Inhibit Pro-inflammatory Cytokine Secretion in Human THP-1 Macrophages and During Primary Biliary Cholangitis
Chronic liver diseases, e.g., cholestasis, are negatively impacted by inflammation, which further aggravates liver injury. Pharmacotherapy targeting the peroxisome proliferator-activated receptor alpha (PPARα), e.g., fenofibrate, has recently become an off-label therapeutic option for patients with refractory cholestasis. Clinical studies show that fibrates can reduce some pro-inflammatory cytokines in primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC); however, its...
15.07.2022 12:00
Primary Sclerosing Cholangitis and Inflammatory Bowel Disease: A Review
Primary sclerosing cholangitis is a disease affecting around 0.006-0.016% of the population. Of these, around 75% have concomitant inflammatory bowel disease (IBD) according to the most recent epidemiological studies. Several theories have been proposed regarding the pathogenesis of primary sclerosing cholangitis (PSC). These include changes in the function of cholangiocytes, effects of the gut microbiome, association with specific human leukocyte antigen haplotypes and dysregulation of the...
15.07.2022 12:00
Fenofibrate in primary sclerosing cholangitis; a randomized, double-blind, placebo-controlled trial
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with no medical treatment proven to improve survival and postpone liver transplantation. Previous studies have shown the effectiveness of fibrates in primary biliary cholangitis. The current study prospectively evaluated the effect of fenofibrate on PSC patients. We administered 200 mg of fenofibrate to PSC patients in the intervention arm and a placebo in the control arm once per day for 6 months and evaluated liver...
13.07.2022 12:00
The focus clinical research in intrahepatic cholangiocarcinoma
Intrahepatic cholangiocarcinoma (ICC), highly invasive and highly heterogeneous, has a poor prognosis. It has been confirmed that many risk factors are associated with ICC including intrahepatic lithiasis, primary sclerosing cholangitis (PSC), congenital abnormalities of the bile ducts, parasite infection, toxic exposures chronic liver disease (viral infection and cirrhosis) and metabolic abnormalities. In recent years, significant progress has been made in the clinical diagnosis and treatment...
12.07.2022 12:00
Skin Changes in Cirrhosis
Skin manifestations of systemic disorders give a clue to the organ involved and help identify the possible disease-causing injury. Skin changes of liver cirrhosis are not specific, as they may be seen in disorders not involving the liver. Thus, a constellation of skin changes along with systemic features may help us to identify the disease-causing liver cirrhosis. Pruritus is one of the most common and distressful symptoms of liver cirrhosis, severely affecting the quality of life, which further...
11.07.2022 12:00
A New Strategy of Liver Transplantation for Locally Advanced Unresectable Perihilar Cholangiocarcinoma Using Living Grafts With Simultaneous Resection of Recipients' Hepatic Artery and Portal Vein Without Neoadjuvant Radiation: A Case Report
CONCLUSION: LDLT with neoadjuvant radiation is associated with high risk of vascular complications. In some cases, conversion surgery after radical chemotherapy using good timing LDLT without radiation may increase chances of transplantation for locally advanced pCCA.
09.07.2022 12:00
A Systematic Review and Meta-Analysis on Metabolic Bone Disease in Patients with Primary Sclerosing Cholangitis
Data about the association between primary sclerosing cholangitis (PSC) and metabolic bone disease are still unclear. PSC is a chronic cholestatic liver disease (CCLD) which affects the biliary tract, and it has a highly variable natural history. We systematically searched until 28 February 2022 MEDLINE, Cochrane Central Register of Controlled Trials, the ISI Web of Science, and SCOPUS, for studies in patients with PSC. We identified 343 references to potential studies. After screening them, we...
09.07.2022 12:00
Serological biomarkers for management of primary sclerosing cholangitis
Clinical manifestations and progression of primary sclerosing cholangitis (PSC) are heterogeneous, and its pathogenesis is poorly understood. The importance of gut-liver interactions in the pathogenesis has been clinically confirmed and highlighted in different theories. Recent advances regarding biomarkers of biliary-gut crosstalk may help to identify clinically relevant PSC subgroups assisting everyday clinical work-up (e.g., diagnosis, disease stratification, or surveillance) and the...
08.07.2022 12:00
Mast cells selectively target large cholangiocytes during biliary injury via H2HR-mediated cAMP/pERK1/2 signaling
Bile ducts are heterogenous in structure and function, and primary sclerosing cholangitis (PSC) damages specific bile ducts leading to ductular reaction (DR), mast cell (MC) infiltration, increased histamine release, inflammation, and fibrosis. Bile duct ligation (BDL) induces large duct damage via cyclic adenosine monophosphate (cAMP)/extracellular signal-related protein kinase (ERK) signaling, and large cholangiocytes express H2 histamine receptor (H2HR). We evaluated how MCs interact with...
08.07.2022 12:00
Inflammatory type 2 conventional dendritic cells contribute to murine and human cholangitis
CONCLUSIONS: Cholangitis is characterised by intrahepatic expansion and inflammatory maturation of type 2 conventional dendritic cells in response to biliary injury. Therefore, type 2 conventional dendritic cells and their inflammatory mediators might be potential therapeutic targets for the treatment of PSC.
07.07.2022 12:00
Impact of thiopurines and tumour necrosis factor antagonists on primary sclerosing cholangitis outcomes in patients with inflammatory bowel disease
CONCLUSIONS: Patients with PSC and IBD who are exposed to thiopurines or anti-TNF agents are not at excess risk of biliary tract cancer or liver transplantation.
05.07.2022 12:00
Causal effect of autoimmune liver diseases on cancer: Meta-analyses of cohort studies and Mendelian randomization study
CONCLUSIONS: In addition to hepatobiliary cancer, results from the meta-analyses suggest that patients with AiLD might have an increased risk of several extrahepatobiliary cancers. However, the causal role of AiLD in cancer development needs to be further investigated.
01.07.2022 12:00
Increased Risk of Non-Conventional and Invisible Dysplasias in Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Disease
CONCLUSIONS: Nearly a third of PSC-IBD patients developed dysplasia, which is often associated with non-conventional dysplastic features, invisible endoscopic/gross appearance, right/proximal-sided colon, multifocality, and advanced neoplasia on follow-up. These findings underscore the importance of recognizing these non-conventional subtypes by practicing pathologists and the need for careful and frequent endoscopic surveillance with random biopsies in PSC-IBD patients.
30.06.2022 12:00
TREM-2 plays a protective role in cholestasis by acting as a negative regulator of inflammation
CONCLUSIONS: TREM-2 acts as a negative regulator of inflammation during cholestasis, representing a novel potential therapeutic target.
24.06.2022 12:00
Clinical Utility of Personalized Serum IgG Subclass Ratios for the Differentiation of IgG4-Related Sclerosing Cholangitis (IgG4-SC) from Primary Sclerosing Cholangitis (PSC) and Cholangiocarcinoma (CCA)
CONCLUSIONS: The serum IgG4/IgG1 or IgG4/(IgG1+IgG3) level may help to differentiate IgG4-SC from PSC. IgG4 alone is the most accurate serologic marker for the differentiation of IgG4-SC from CCA.
24.06.2022 12:00
Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics
Cholangiopathies encompass various biliary diseases affecting the biliary epithelium, resulting in cholestasis, inflammation, fibrosis, and ultimately liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults. Much research has broadened the scope of disease biology to genetic risk, epigenetic changes, dysregulated mucosal immunity, altered biliary epithelial cell function, and dysbiosis, all of which...
24.06.2022 12:00
Spatial transcriptomics identifies enriched gene expression and cell types in human liver fibrosis
Liver fibrosis and cirrhosis have limited therapeutic options and represent a serious unmet patient need. Recent use of single-cell RNA sequencing (scRNAseq) has identified enriched cell types infiltrating cirrhotic livers but without defining the microanatomical location of these lineages thoroughly. To assess whether fibrotic liver regions specifically harbor enriched cell types, we explored whether whole-tissue spatial transcriptomics combined with scRNAseq and gene deconvolution analysis...
21.06.2022 12:00
Digital single-operator video cholangioscopy improves endoscopic management in patients with primary sclerosing cholangitis-a retrospective observational study
CONCLUSION: Digital SOVC is effective and safe for the endoscopic management of PSC patients and may be regularly considered an additive tool for the complex endoscopic management of these patients.
20.06.2022 12:00
Protective and aggressive bacterial subsets and metabolites modify hepatobiliary inflammation and fibrosis in a murine model of PSC
CONCLUSIONS: We identified novel functionally protective and detrimental resident bacterial species in mdr2^(-/-) mice and PSC patients with associated clinical risk score. These insights may guide personalised targeted therapeutic interventions in PSC patients.
15.06.2022 12:00
Immune checkpoint inhibitor-related cholangiopathy: Novel clinicopathological description of a multi-centre cohort
CONCLUSION: Within this heterogeneous cohort, we identified that CPI-related cholangiopathy responded poorly to immunosuppression and potentially progressed to bile duct loss. Thorough radiological and histological assessment is recommended, as identification of the cholangiopathy-associated phenotype may permit more informed advice regarding prognosis. Further data is required to determine detailed immunological characterisation in order to identify individuals at an increased risk of...
15.06.2022 12:00
Anti-Proteinase 3 Antibodies as a Biomarker for Ulcerative Colitis and Primary Sclerosing Cholangitis in Children
CONCLUSIONS: Our study provides data for PR3-ANCA as a potential serological marker for paediatric UC and PSC.
15.06.2022 12:00
Pericholangitis
Primary sclerosing cholangitis (PSC) is an uncommon cholestatic liver disease characterized by the inflammation and fibrosis of intra- and extra-hepatic biliary ducts. It has an incidence of 0.9 to 1.3 cases per 100000 in the United States. After recruiting of endoscopic retrograde cholangiopancreatography (ERCP) in the early 1970s, it revealed that ‘classical’ primary sclerosing cholangitis (PSC) appears as multiple strictures in the intra- and extra-hepatic biliary tree. However, the ERCP is...
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