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Primary Sclerosing Cholangitis

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2. BSG and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis

BSG and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis 1 Chapman MH, et al. Gut 2019;66:1–23. doi:10.1136/gutjnl-2018-317993 Guidelines British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis Michael Huw Chapman, 1,2 Douglas Thorburn, 2 Gideon M Hirschfield, 3 George G J Webster, 1 Simon M Rushbrook, 4 Graeme Alexander, 2 Jane Collier, 5 Jessica K Dyson, 6,7 David EJ Jones, 7 Imran (...) Pereira; stephen. pereir a@ ucl . ac. uk Received 25 November 2018 Revised 21 February 2019 Accepted 24 March 2019 © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. AbsTrACT These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology

2019 British Society of Gastroenterology

3. Primary sclerosing cholangitis

Primary sclerosing cholangitis Primary sclerosing cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Primary sclerosing cholangitis Last reviewed: February 2019 Last updated: November 2017 Summary Cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease. Predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease

2017 BMJ Best Practice

4. Follicular T Helper Cell Signatures in Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Full Text available with Trip Pro

Follicular T Helper Cell Signatures in Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are the most common cholestatic liver diseases. While PBC is generally accepted to be an autoimmune disorder characterized by pathognomonic autoantibodies against mitochondrial antigens, the pathogenesis of PSC is less precisely defined; however, some degree of altered immunity toward autoantigens has been suggested

2018 Hepatology communications

5. Role of endoscopy in primary sclerosing cholangitis

Role of endoscopy in primary sclerosing cholangitis Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline – European Society of Gastrointestinal Endoscopy (ESGE) +49-89-9077936-11 Menu DOI: 10.1055/s-0043-107029 Endoscopy 2017; 49(06): 588-608 © Georg Thieme Verlag KG Stuttgart· New York Explore the ESGE website Menu Contact us +49-89-9077936-0 Follow us © 2004

2017 European Society of Gastrointestinal Endoscopy

6. Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. Full Text available with Trip Pro

Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. Cholestatic liver diseases encompass a broad spectrum of pathologies, with the core injury occurring at the level of cholangiocytes and progressing to hepatic fibrosis and liver dysfunction. Primary biliary cholangitis and primary sclerosing cholangitis are the most significant progressive cholangiopathies in adults. Although rare, they commonly evolve to liver failure and need for liver transplantation. Despite recent advances

2019 American Journal of Gastroenterology

7. Searching for Bacterial Biofilm in Recurrent Cholangitis in Primary Sclerosing Cholangitis: A Case Presentation and Introduction of an Unexplored Disease Mechanism Full Text available with Trip Pro

Searching for Bacterial Biofilm in Recurrent Cholangitis in Primary Sclerosing Cholangitis: A Case Presentation and Introduction of an Unexplored Disease Mechanism Inflammation and fibrosis of the bile ducts are the defining pathological characteristics of primary sclerosing cholangitis (PSC). A previously unexplored mechanism for recurrent cholangitis, one of PSC's most common presentations, is bacterial colonization of the biliary epithelium in the form of biofilm, which may confer resistance (...) to antibiotics and host phagocytic machinery. The aim of the current study was to assess whether bacteria could be seen on the liver explant and whether they organized in the form of biofilm. An explanted PSC liver from a 60-year-old male who suffered from recurrent cholangitis was formalin-fixed, paraffin-embedded and Gram stained. The specimens were observed under light microscopy. Neither bacteria nor biofilm were detected. We did not detect bacteria or biofilm in the liver explant of a single PSC patient

2017 Journal of clinical and translational hepatology

8. Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis Full Text available with Trip Pro

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis Adverse clinical events in primary sclerosing cholangitis (PSC) happen too slowly to capture during clinical trials. Surrogate endpoints are needed, but no such validated endpoints exist for children with PSC. We evaluated the association between gamma glutamyltransferase (GGT) reduction and long-term outcomes in pediatric PSC patients. We evaluated GGT normalization (< 50 IU/L

2018 Hepatology communications

9. Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices Full Text available with Trip Pro

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices Dominant strictures (DSs) of the biliary tree occur in approximately 50% of patients with primary sclerosing cholangitis (PSC) and may cause significant morbidity. Nevertheless, the definition and management of DSs lacks consensus. We aimed to better understand current perceptions and practices regarding PSC-associated DSs. We conducted an anonymous, 23-question, survey-based study

2018 Hepatology communications

10. Overlap Syndrome between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Full Text available with Trip Pro

Overlap Syndrome between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Overlap syndrome indicates the coexistence of 2 or more autoimmune liver diseases in the same individual, occurring simultaneously or sequentially. Cases of overlap of autoimmune hepatitis (AIH) with primary biliary cholangitis (PBC) and of AIH with primary sclerosing cholangitis (PSC) are known and have defined criteria for diagnosis. Overlap between PBC and PSC has been reported in only a few case reports

2018 ACG case reports journal

11. Non-invasive Prediction of High-risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. Full Text available with Trip Pro

Non-invasive Prediction of High-risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. Baveno-VI guidelines recommend that patients with compensated cirrhosis with liver stiffness by transient elastography (LSM-TE) <20 kPa and platelets >150,000/mm do not need an esophagogastroduodenoscopy (EGD) to screen for varices, since the risk of having varices needing treatment (VNT) is <5%. It remains uncertain if this tool can be used in patients with cholestatic (...) liver diseases (ChLDs): primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). These patients may have a pre-sinusoidal component of portal hypertension that could affect the performance of this rule. In this study we evaluated the performance of Baveno-VI, expanded Baveno-VI (LSM-TE <25 kPa and platelets >110,000/mm), and other criteria in predicting the absence of VNT.This was a multicenter cross-sectional study in four referral hospitals. We retrospectively analyzed data from

2018 American Journal of Gastroenterology

12. No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis Full Text available with Trip Pro

No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis Dominant strictures occur in approximately 50% of patients with primary sclerosing cholangitis (PSC). Short-term stents have been reported to produce longer resolution of dominant strictures than single-balloon dilatation. We performed a prospective study to compare the efficacy and safety of balloon dilatation vs short-term stents in patients with non-end-stage PSC.We (...) performed an open-label trial of patients with PSC undergoing therapeutic endoscopic retrograde cholangiopancreatography (ERCP) at 9 tertiary-care centers in Europe, from July 2011 through April 2016. Patients found to have a dominant stricture during ERCP were randomly assigned to groups that underwent balloon dilatation (n = 31) or stent placement for a maximum of 2 weeks (n = 34); patients were followed for 24 months. The primary outcome was the cumulative recurrence-free patency of the primary

2018 EvidenceUpdates

13. Primary sclerosing cholangitis. (Abstract)

Primary sclerosing cholangitis. Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal (...) hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance

2018 Lancet

14. Changes in Liver Stiffness, Measured by Magnetic Resonance Elastography, Associated With Hepatic Decompensation in Patients With Primary Sclerosing Cholangitis. Full Text available with Trip Pro

Changes in Liver Stiffness, Measured by Magnetic Resonance Elastography, Associated With Hepatic Decompensation in Patients With Primary Sclerosing Cholangitis. Single measurements of liver stiffness (LS) by magnetic resonance elastography (MRE) have been associated with outcomes of patients with primary sclerosing cholangitis (PSC), but the significance of changes in LS over time are unclear. We investigated associations between changes in LS measurement and progression of PSC.We performed (...) a retrospective review of 204 patients with patients who underwent 2 MREs at a single center between January 1, 2007 and December 31, 2018. We collected laboratory data and information on revised Mayo PSC risk and model for end-stage liver disease scores, the PSC risk estimate tool, and levels of aspartate transferase at the time of each MRE. The ΔLS/time was determined by the change in LS between the second MRE compared to the first MRE divided by the time between examinations. The primary endpoint

2020 Clinical Gastroenterology and Hepatology

15. Genetic variants of UDP-glucuronosyltransferase 1A genes are associated with disease presentation and outcome in primary sclerosing cholangitis. Full Text available with Trip Pro

Genetic variants of UDP-glucuronosyltransferase 1A genes are associated with disease presentation and outcome in primary sclerosing cholangitis. Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease without a curative medical therapy. The human UDP-glucuronosyltransferases 1A play a major role in the detoxification and elimination of bilirubin, bile acids and xenobiotics. Whether genetic UGT1A variants determine course and outcome of PSC has not yet been described.A

2020 Liver International

16. Hepatocyte KLF6 expression affects FXR signalling and the clinical course of primary sclerosing cholangitis. Full Text available with Trip Pro

Hepatocyte KLF6 expression affects FXR signalling and the clinical course of primary sclerosing cholangitis. Primary sclerosing cholangitis (PSC) is characterized by chronic cholestasis and inflammation, which promotes cirrhosis and an increased risk of cholangiocellular carcinoma (CCA). The transcription factor Krueppel-like-factor-6 (KLF6) is a mediator of liver regeneration, steatosis, and hepatocellular carcinoma (HCC), but no data is yet available on its potential role in cholestasis. Here

2020 Liver International

17. Effects of Tumor Necrosis Factor Antagonists in Patients With Primary Sclerosing Cholangitis. (Abstract)

Effects of Tumor Necrosis Factor Antagonists in Patients With Primary Sclerosing Cholangitis. Few patients with primary sclerosing cholangitis (PSC) and inflammatory bowel diseases (IBDs) are exposed to tumor necrosis factor (TNF) antagonists because of the often mild symptoms of IBD. We assessed the effects of anti-TNF agents on liver function in patients with PSC and IBD, and their efficacy in treatment of IBD.We performed a retrospective analysis of 141 patients with PSC and IBD receiving (...) associated significantly with level of ALP during anti-TNF therapy.Anti-TNF treatment produced a response of IBD in 48% of patients and remission of IBD in 23%. There was no difference in PSC symptom frequency before or after drug exposure. The most common reasons for anti-TNF discontinuation were primary nonresponse of IBD (17%) and side effects (18%). At 3 months, infliximab-treated patients had a median reduction in serum level of ALP of 4% (interquartile range, reduction of 25% to increase of 19

2020 Clinical Gastroenterology and Hepatology

18. Risk of gallbladder cancer in patients with primary sclerosing cholangitis and radiographically detected gallbladder polyps. (Abstract)

Risk of gallbladder cancer in patients with primary sclerosing cholangitis and radiographically detected gallbladder polyps. Primary sclerosing cholangitis (PSC) is associated with an increased risk of gallbladder cancer (GBC). Gallbladder polyps potentially harbour malignancy and thus international guidelines recommend prophylactic cholecystectomy for gallbladder polyps of any size in patients with PSC. To best inform patient care we sought to quantify the malignant risk of gallbladder polyps

2020 Liver International

19. Assessing the Validity of Adult-derived Prognostic Models for Primary Sclerosing Cholangitis Outcomes in Children. (Abstract)

Assessing the Validity of Adult-derived Prognostic Models for Primary Sclerosing Cholangitis Outcomes in Children. Natural history models for primary sclerosing cholangitis (PSC) are derived from adult patient data, but have never been validated in children. It is unclear how accurate such models are for children with PSC.We utilized the pediatric PSC consortium database to assess the Revised Mayo Clinic, Amsterdam-Oxford, and Boberg models. We calculated the risk stratum and predicted survival

2020 Journal of Pediatric Gastroenterology and Nutrition

20. Letter to the Editors: Could Butyrate Be Incorporated With Farnesoid X Receptor Agonist Cilofexor to Enhance Primary Sclerosing Cholangitis Treatment? (Abstract)

Letter to the Editors: Could Butyrate Be Incorporated With Farnesoid X Receptor Agonist Cilofexor to Enhance Primary Sclerosing Cholangitis Treatment? Primary sclerosing cholangitis (PSC) is characterized by chronic liver inflammation and cholestasis due to intrahepatic and extrahepatic bile duct inflammation and fibrosis. Cholestasis results in accumulation of bile acids in the liver with a concomitant deficit in the intestines. Accumulation of bile acids could be causal for inflammation

2020 Hepatology

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