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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 (PubMed)

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

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2018 Hepatology communications

5. Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. (PubMed)

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

6. Searching for Bacterial Biofilm in Recurrent Cholangitis in Primary Sclerosing Cholangitis: A Case Presentation and Introduction of an Unexplored Disease Mechanism (PubMed)

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

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2017 Journal of clinical and translational hepatology

7. Overlap Syndrome between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis (PubMed)

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

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2018 ACG case reports journal

8. Non-invasive Prediction of High-risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. (PubMed)

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

9. Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices (PubMed)

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

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2018 Hepatology communications

10. Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis (PubMed)

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

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2018 Hepatology communications

11. Primary sclerosing cholangitis. (PubMed)

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

12. No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis

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. Stent versus balloon dilation for the treatment of dominant strictures in primary sclerosing cholangitis: a systematic review and meta-analysis

Stent versus balloon dilation for the treatment of dominant strictures in primary sclerosing cholangitis: a systematic review and meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content (...) . Not an in vivo animal study 3. No metastases/ only primary tumor 4. No control group 5. Combination therapy or contamination 6. Not about analgesics used in the clinic Full text-screening: As above, with the addition of: 7. No relevant outcome measure reported ">Prioritise the exclusion criteria Example: Two reviewers will independently extract data from each article. We first try to extract numerical data from tables, text or figures. If these are not reported, we will extract data from graphs using digital

2019 PROSPERO

14. Epigenetics in the Primary Biliary Cholangitis and Primary Sclerosing Cholangitis (PubMed)

Epigenetics in the Primary Biliary Cholangitis and Primary Sclerosing Cholangitis 28564724 2018 03 20 2018 11 13 1098-8971 37 2 2017 05 Seminars in liver disease Semin. Liver Dis. Epigenetics in the Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. 159-174 10.1055/s-0037-1603324 Cheung Angela C AC Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota. LaRusso Nicholas F NF Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota (...) , Non-U.S. Gov't 2017 05 31 United States Semin Liver Dis 8110297 0272-8087 0 Genetic Markers 0 Histones 0 RNA, Untranslated IM Animals Cholangitis, Sclerosing diagnosis genetics metabolism Chromatin Assembly and Disassembly DNA Methylation Epigenesis, Genetic Genetic Markers Genetic Predisposition to Disease Genome-Wide Association Study Heredity Histones genetics metabolism Humans Liver Cirrhosis, Biliary diagnosis genetics metabolism Pedigree Phenotype RNA, Untranslated genetics metabolism Risk

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2017 Seminars in Liver Disease

15. A novel approach to genome-wide association analysis identifies genetic associations with primary biliary cholangitis and primary sclerosing cholangitis in Polish patients (PubMed)

A novel approach to genome-wide association analysis identifies genetic associations with primary biliary cholangitis and primary sclerosing cholangitis in Polish patients Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are forms of hepatic autoimmunity, and risk for both diseases has a strong genetic component. This study aimed to define the genetic architecture of PBC and PSC within the Polish population.Subjects were 443 women with PBC, 120 patients with PSC

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2017 BMC medical genomics

16. Cholangiocarcinoma is associated with a raised enhanced liver fibrosis (ELF) score independent of primary sclerosing cholangitis. (PubMed)

Cholangiocarcinoma is associated with a raised enhanced liver fibrosis (ELF) score independent of primary sclerosing cholangitis. Cholangiocarcinoma (CCA) complicates primary sclerosing cholangitis (PSC) in 10-20% of cases, but current tools for prediction of a CCA diagnosis are inadequate. Recently we demonstrated the utility of the ELF test to stratify prognosis in PSC. We observed that patients with PSC+CCA had significantly higher ELF score than those with PSC alone. In this study, we aimed

2019 European journal of clinical investigation

17. Longitudinal analysis of CA19-9 reveals individualised normal range and early changes before development of biliary tract cancer in patients with primary sclerosing cholangitis. (PubMed)

Longitudinal analysis of CA19-9 reveals individualised normal range and early changes before development of biliary tract cancer in patients with primary sclerosing cholangitis. Primary sclerosing cholangitis is associated with an increased risk of biliary tract cancer. Carbohydrate antigen 19-9 (CA19-9) can be used to screen for these malignancies.To perform a longitudinal analysis of CA19-9 in patients with primary sclerosing cholangitis.We conducted a retrospective analysis of CA19-9 values (...) in patients who had primary sclerosing cholangitis, with and without biliary malignancy. We calculated the index of individuality and reference change value in patients who were cancer-free. Long-term analysis of CA19-9 in cancer-free patients was performed and we assessed the change of CA19-9 prior to diagnosis of cancer.We obtained 1818 CA19-9 values from 247 patients, including 32 with malignancy. Median CA19-9 in cancer-free individuals was 15.6 U/mL. The index of individuality was 0.37

2019 Alimentary Pharmacology & Therapeutics

18. Th17 cell frequency is associated with low bone mass in primary sclerosing cholangitis. (PubMed)

Th17 cell frequency is associated with low bone mass in primary sclerosing cholangitis. Osteoporotic fractures are a major cause of morbidity and reduced quality of life in patients with primary sclerosing cholangitis (PSC), a progressive bile duct disease of unknown origin. Although it is generally assumed that this pathology is a consequence of impaired calcium homeostasis and malabsorption, the cellular and molecular causes of PSC-associated osteoporosis are unknown.We determined bone (...) analyzed the skeletal phenotype of mice lacking Abcb4 and/or Il-17.Unlike in patients with primary biliary cholangitis, bone loss in patients with PSC was not associated with disease duration or liver fibrosis. However, we observed a significant negative correlation between the bone resorption biomarker deoxypyridinoline and bone mineral density in the PSC cohort, indicating increased bone resorption. Importantly, the frequency of Th17 cells in peripheral blood was positively correlated

2019 Journal of Hepatology

19. A novel prognostic model for transplant-free survival in primary sclerosing cholangitis

A novel prognostic model for transplant-free survival in primary sclerosing cholangitis Most prognostic models for primary sclerosing cholangitis (PSC) are based on patients referred to tertiary care and may not be applicable for the majority of patients with PSC. The aim of this study was to construct and externally validate a novel, broadly applicable prognostic model for transplant-free survival in PSC, based on a large, predominantly population-based cohort using readily available

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2017 EvidenceUpdates

20. Association of gadolinium-enhanced magnetic resonance imaging with hepatic fibrosis and inflammation in primary sclerosing cholangitis. (PubMed)

Association of gadolinium-enhanced magnetic resonance imaging with hepatic fibrosis and inflammation in primary sclerosing cholangitis. To evaluate magnetic resonance imaging (MRI) parameters T2 signal, contrast enhancement (CE), and relative liver enhancement (RLE) of extracellular gadolinium-based contrast agent (GBCA)-enhanced MRI as a marker for hepatic fibrosis and inflammation in patients with primary sclerosing cholangitis (PSC).3.0-Tesla MRI scans and liver biopsies of 40 patients (41.2

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2018 PLoS ONE

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