6,992 results match your criteria Primary Sclerosing Cholangitis


Diagnosing Primary Sclerosing Cholangitis in Children.

Cureus 2020 May 28;12(5):e8334. Epub 2020 May 28.

Radiology, Pakistan Kidney and Liver Institute and Research Center, Lahore, PAK.

Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease in children caused by chronic inflammatory process affecting either intrahepatic or extrahepatic bile ducts. Although it is infrequent, incidence is increasing worldwide, may be due to more awareness, understanding and expertise in managing children with chronic liver disease (CLD). In the developing world like Pakistan where resources and expertise are limited, very few tertiary centers are equipped to manage CLD in children. Read More

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http://dx.doi.org/10.7759/cureus.8334DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7325345PMC

Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan.

J Hepatobiliary Pancreat Sci 2020 Jun 30. Epub 2020 Jun 30.

The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Japan.

Background/purpose: Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce.

Method: We randomly selected healthcare facilities from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. Read More

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http://dx.doi.org/10.1002/jhbp.793DOI Listing

Impact of Pre-existing Inflammatory Bowel Disease on the Outcome of Liver Transplantation for Primary Sclerosing Cholangitis.

Liver Transpl 2020 Jun 30. Epub 2020 Jun 30.

Service d'hépatologie, Hôpital Haut Lévêque, CHU de Bordeaux, France.

Approximately 80% of patients with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD). The effect of IBD on the outcomes of liver transplantation (LT) for PSC is unclear. We retrospectively collected data from adults who underwent LT for PSC from 1989 to 2017 in four French LT centers. Read More

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http://dx.doi.org/10.1002/lt.25838DOI Listing

A Patient with Primary Sclerosing Cholangitis and Diffuse Biliary Dilation.

Gastroenterology 2020 Jun 26. Epub 2020 Jun 26.

Vatche and Tamar Manoukian Division of Digestive Diseases, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.

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http://dx.doi.org/10.1053/j.gastro.2020.06.053DOI Listing

Challenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1.

Therap Adv Gastroenterol 2020 16;13:1756284820927292. Epub 2020 Jun 16.

Department of Medicine, Geneva University, Geneva, Switzerland.

It is frequently challenging to make the correct diagnosis in patients with biliary strictures. This is particularly important as errors may have disastrous consequences. Benign-appearing strictures treated with stents may later be revealed to be malignant and unnecessary surgery for benign strictures carries a high morbidity rate. Read More

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http://dx.doi.org/10.1177/1756284820927292DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7298429PMC

The Potentiality of Herbal Remedies in Primary Sclerosing Cholangitis: From to Clinical Studies.

Front Pharmacol 2020 10;11:813. Epub 2020 Jun 10.

Institute of Clinical Physiology, National Research Council (CNR), Pisa, Italy.

Primary sclerosing cholangitis is a complex pathological condition, characterized by chronic inflammation and fibrosis of the biliary epithelium. Without proper clinical management, progressive bile ducts and liver damage lead to cirrhosis and, ultimately, to liver failure. The known limited role of current drugs for treating this cholangiopathy has driven researchers to assess alternative therapeutic options. Read More

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http://dx.doi.org/10.3389/fphar.2020.00813DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7298067PMC

Incidence, Risk Factors, and Outcomes of Pouchitis and Pouch-related Complications in Patients with Ulcerative Colitis.

Clin Gastroenterol Hepatol 2020 Jun 22. Epub 2020 Jun 22.

Division of Gastroenterology and Hepatology, University of North Carolina at Chapel Hill, Chapel Hill, NC; Center for Gastrointestinal Biology and Disease, University of North Carolina at Chapel Hill, Chapel Hill, NC.

Background & Aims: Acute pouchitis is the most common non-surgical complication after restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC). We used validated case-finding definitions for pouchitis to search administrative claims data and determine the incidence of pouchitis in the first 2 years after IPAA.

Methods: We identified all patients who underwent proctocolectomy with IPAA for UC in the IQVIA Legacy PharMetrics Adjudicated Claims Database, from January 1, 2007 through June 1, 2016. Read More

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http://dx.doi.org/10.1016/j.cgh.2020.06.035DOI Listing

Risks and Characteristics of Pancreatic Cancer and Pancreatic Relapse in Autoimmune Pancreatitis Patients.

J Gastroenterol Hepatol 2020 Jun 24. Epub 2020 Jun 24.

Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Background And Aim: We examined the differences in the risks and characteristics of pancreatic relapse (PR) and pancreatic cancer (PC) in patients with autoimmune pancreatitis (AIP).

Methods: We retrospectively reviewed 123 type 1 AIP patients with a median follow-up of 55 months (interquartile range, 27-98). The following items were evaluated: 1) cumulative relapse rates and risk factors, 2) the incidence of PC, 3) PR versus PC, and 4) outcomes after the appearance of morphological changes in the pancreas (focal enlargement, apparent mass lesions, or main pancreatic duct dilation [MPDD]). Read More

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http://dx.doi.org/10.1111/jgh.15163DOI Listing

An Overlapping Case of IgG4-related Disease and Klinefelter Syndrome with Lupus-like Serological and Neurological Features: A Case Report and Literature Review.

Intern Med 2020 Jun 23. Epub 2020 Jun 23.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Japan.

A 46-year-old man with Klinefelter syndrome (KS) presented with obliterative phlebitis of the lower legs with a deteriorated renal function, and elevated serum alkaline phosphatase and ataxia levels. Examinations demonstrated tubulointerstitial nephritis, obliterative phlebitis and lymphadenopathy with IgG4+ plasma cell infiltrate and sclerosing cholangitis. Although the serological profile and central nerve system involvement were compatible for systemic lupus erythematosus (SLE), a definite diagnosis of SLE was difficult to make. Read More

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http://dx.doi.org/10.2169/internalmedicine.4888-20DOI Listing

Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single-center experience.

Pediatr Transplant 2020 Jun 23:e13768. Epub 2020 Jun 23.

Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Antalya, Turkey.

Deficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoprotein CD40L (CD154) gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality. Read More

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http://dx.doi.org/10.1111/petr.13768DOI Listing

Contribution of the Intestinal Microbiome and Gut Barrier to Hepatic Disorders.

Gastroenterology 2020 Jun 19. Epub 2020 Jun 19.

Emory Vaccine Center, Division of Microbiology and Immunology, Yerkes National Primate Research Center, Emory University School of Medicine, Atlanta, GA; Division of Infectious Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, GA. Electronic address:

Intestinal barrier dysfunction and dysbiosis contribute to development of diseases in liver and other organs. Physical, immunological, and microbiologic (bacterial, fungal, archaeal, viral, and protozoal) features of the intestine separate its nearly one hundred trillion microbes from the rest of the human body. Failure of any aspect of this barrier can result in translocation of microbes into the blood and sustained inflammatory response that promote liver injury, fibrosis, cirrhosis, and oncogenic transformation. Read More

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http://dx.doi.org/10.1053/j.gastro.2020.04.077DOI Listing

A rare presentation of hepatolithiasis in an adolescent patient: A case report.

Int J Surg Case Rep 2020 Jun 12;72:343-345. Epub 2020 Jun 12.

Department of Surgery, Division of Surgical Oncology, University of California San Francisco, San Francisco, CA, USA. Electronic address:

Introduction: Hepatolithiasis (intrahepatic stones) is rare in adolescent patients and requires complex management strategies to prevent recurrent infections and progression to hepatic fibrosis. Surgical management is often required. In cases of unclear etiology, further work-up is indicated to provide insight into future management. Read More

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http://dx.doi.org/10.1016/j.ijscr.2020.06.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7306511PMC

Senescent Cholangiocytes Release Extracellular Vesicles that Alter Target Cell Phenotype Via the Epidermal Growth Factor Receptor.

Liver Int 2020 Jun 18. Epub 2020 Jun 18.

Division of Gastroenterology and Hepatology, Mayo Clinic Center for Cell Signaling in Gastroenterology, Mayo Clinic, Rochester, Minnesota, 55905, USA.

Background & Aims: Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by peribiliary inflammation and fibrosis. Cholangiocyte senescence is a prominent feature of PSC. Here we hypothesize that extracellular vesicles (EVs) from senescent cholangiocytes influence the phenotype of target cells. Read More

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http://dx.doi.org/10.1111/liv.14569DOI Listing

Use of Butorphanol as Treatment for Cholestatic Itch.

Dig Dis Sci 2020 Jun 18. Epub 2020 Jun 18.

Division of Hepatology, Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, 1500 NW 12th Ave Ste 1101, Miami, FL, USA.

Background: Pruritus is a debilitating symptom of cholestatic diseases such as primary biliary cholangitis and primary sclerosing cholangitis and often results in major reduction in quality of life for afflicted patients. Classic treatment options for the treatment of cholestatic pruritus include antihistamines, bile acid resins, serotonin reuptake inhibitors, and mu-opioid antagonists. Unfortunately, these drugs are not always successful in treating pruritus of cholestasis and may be associated with adverse effects. Read More

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http://dx.doi.org/10.1007/s10620-020-06392-2DOI Listing

Comparison of Coagulation Conditions in Patients With Liver Cirrhosis Due to Primary Sclerosing Cholangitis and Nonbiliary Causes of Cirrhosis Before Orthotopic Liver Transplant.

Exp Clin Transplant 2020 Jun 16. Epub 2020 Jun 16.

>From the Liver Transplantation Research Center, Imam Khomeini Hospital Complex, and the Department of Anesthesiology and Critical Care, Tehran University of Medical Sciences, Sina Hospital, Tehran, Iran.

Objectives: Orthotopic liver transplant can be accompanied by an obscure bleeding pattern in patients with severe hepatic malfunction. In the present study, coagulation conditions of patients with cirrhosis of the liver due to primary sclerosing cholangitis and nonbiliary causes of cirrhosis were compared using rotational thromboelastometry assays obtained before orthotopic liver transplant.

Materials And Methods: This case control study analyzed patients who were candidates for orthotopic liver transplant from 2010 to 2016. Read More

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http://dx.doi.org/10.6002/ect.2018.0374DOI Listing

Gut microbiome in primary sclerosing cholangitis: A review.

World J Gastroenterol 2020 Jun;26(21):2768-2780

Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by biliary inflammation and stricturing. Exploration of the pathogenesis of PSC in light of its association with inflammatory bowel disease (IBD) and the "gut-liver" axis is an emerging area of interest. A growing number of studies have begun to elucidate the role of the gut microbiota, its metabolites and its influence on host immune responses in the development of PSC and PSC-IBD. Read More

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http://dx.doi.org/10.3748/wjg.v26.i21.2768DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284173PMC

Langerhans cell histiocytosis confined to extrahepatic bile duct causing sclerosing cholangitis in child: a case report.

Surg Case Rep 2020 Jun 16;6(1):137. Epub 2020 Jun 16.

Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1, Sakuragaoka, Kagoshima City, 890-8520, Japan.

Background: Langerhans cell histiocytosis (LCH) is an abnormal accumulation of Langerhans cells in various organs that sometimes induces organ dysfunction. LCH can affect the liver, resulting in sclerosing cholangitis and biliary cirrhosis. However, liver and bile duct involvement is usually observed in the disseminated form of LCH. Read More

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http://dx.doi.org/10.1186/s40792-020-00899-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297897PMC

Glycomic analysis of antibody indicates distinctive glycosylation profile in patients with autoimmune cholangitis.

J Autoimmun 2020 Jun 13:102503. Epub 2020 Jun 13.

Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, CA, 95616, USA. Electronic address:

Glycosylation of antibodies, particularly in the Fc domain, critically modulate the ability of antibodies to bind to FcRs, maintaining immune quiescence to achieve a finely orchestrated immune response. The removal of sialic acid and galactose residues dramatically alters the physiological function of IgGs, and alterations of Ig glycosylation have been associated with several autoimmune disorders. However, Ig glycosylation has not been extensively studied in autoimmune cholangitis. Read More

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http://dx.doi.org/10.1016/j.jaut.2020.102503DOI Listing

CTLA-4 Expression Plays a Role in PSC and PBC Progression.

Diseases 2020 Jun 12;8(2). Epub 2020 Jun 12.

Department of Gastroenterology and Hepatology, University Hospital Essen, 45147 Essen, Germany.

Background & Aims: The pathogenesis of primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) remains unclear. The aim of this study was to reveal certain single nucleotide polymorphisms (SNP) in genes for regulatory proteins in the immunologic pathway possibly going along with susceptibility of attaining PBC or PSC.

Methods: 126 patients with either PBC or PSC with clinical and laboratory data were enrolled in the study. Read More

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http://dx.doi.org/10.3390/diseases8020021DOI Listing

Nonalcoholic Steatohepatitis is the Most Rapidly Increasing Indication for Liver Transplantation in the United States.

Clin Gastroenterol Hepatol 2020 Jun 9. Epub 2020 Jun 9.

Center for Outcomes Research in Liver Diseases, Washington, DC.

Background & Aims: The profile of chronic liver disease (CLD) in the United States has changed due to obesity trends and advances in treatment of viral hepatitis. We assessed liver transplant listing trends by CLD etiology.

Methods: Adult candidates for liver transplantation were selected from the Scientific Registry of Transplant Recipients (2002 through 2019). Read More

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http://dx.doi.org/10.1016/j.cgh.2020.05.064DOI Listing
June 2020
7.896 Impact Factor

A 1-Year Cross-sectional Inflammatory Bowel Disease Surveillance Colonoscopy Cohort Comparing High-definition White Light Endoscopy and Chromoendoscopy.

Inflamm Bowel Dis 2020 Jun 12. Epub 2020 Jun 12.

Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, Minnesota, USA.

Background: We sought to compare the dysplasia detection rate of high-definition white light endoscopy (HDWLE) with that of chromoendoscopy in patients with long-standing inflammatory bowel disease (IBD).

Methods: This is a retrospective observational cohort of patients with IBD who underwent surveillance colonoscopy between October 1, 2016 and September 30, 2017. We assessed the association between dysplasia detection and multiple variables. Read More

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http://dx.doi.org/10.1093/ibd/izaa146DOI Listing

Trends in Incidence of Autoimmune Liver Diseases and Increasing Incidence of Autoimmune Hepatitis.

Clin Gastroenterol Hepatol 2020 Jun 8. Epub 2020 Jun 8.

Department of Gastroenterology, Christchurch Hospital, Christchurch, New Zealand; University of Otago, Christchurch, New Zealand. Electronic address:

Background & Aims: Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are autoimmune liver diseases of unknown etiology. We studied trends in incidences of AIH, PBC, and PSC in a population-based prospective study Canterbury, New Zealand.

Methods: We collected data on patients with AIH (n=99), PBC (n=26), or PSC (n=47) from public hospitals and private practices in Canterbury from 2008 through 2016. Read More

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http://dx.doi.org/10.1016/j.cgh.2020.05.061DOI Listing

Patient and clinician opinions of patient reported outcome measures (PROMs) in the management of patients with rare diseases: a qualitative study.

Health Qual Life Outcomes 2020 Jun 10;18(1):177. Epub 2020 Jun 10.

Centre for Patient-Reported Outcomes Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Background: Rare diseases may be life-threatening or chronically debilitating conditions. Patient care needs are often complex and challenging to coordinate and deliver effectively. Rare diseases and their clinical management may therefore substantially impact on patients' health-related quality of life (HRQOL). Read More

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http://dx.doi.org/10.1186/s12955-020-01438-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7288678PMC

Liver disease referrals to an urban, hospital-based hepatology outpatient clinic over the past 25 years.

JGH Open 2020 Jun 5;4(3):484-489. Epub 2019 Dec 5.

Section of Hepatology, Department of Internal Medicine University of Manitoba Winnipeg Manitoba Canada.

Background: Additional hepatologists are required to manage the rapidly increasing number of patients with liver disease. One disincentive to trainees considering a career in hepatology is the longstanding perception that outpatient hepatology consists largely of managing patients with alcohol-induced liver disease (ALD).

Objectives: To document the types of liver diseases and changes in liver disease referrals to an urban outpatient liver disease clinic over the past 25 years. Read More

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http://dx.doi.org/10.1002/jgh3.12286DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7273706PMC

Liver Elastography in Primary Sclerosing Cholangitis Patients Using Three Different Scanner Systems.

Ultrasound Med Biol 2020 Aug 2;46(8):1854-1864. Epub 2020 Jun 2.

National Centre for Ultrasound in Gastroenterology, Department of Medicine, Haukeland University Hospital, Bergen, Norway; Department of Medicine, Haraldsplass Deaconess Hospital, Bergen, Norway; Norwegian PSC Research Center (NoPSC), Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Department of Clinical Science, University of Bergen, Bergen, Norway.

The aim of the study described here was to characterize three different liver elastography methods in primary sclerosing cholangitis (PSC) patients, for the first time exploring 2-D shear wave elastography (2-D-SWE) in PSC patients and its putative advantages over point shear wave elastography (pSWE). Sixty-six adult PSC patients (51 males, 77%) underwent liver elastography: Transient elastography (TE), pSWE and 2-D-SWE were applied head-to-head after B-mode ultrasonography and blood tests. Liver stiffness measurements (LSMs) by pSWE yielded lower values than those by TE; 2-D-SWE had less steep slope but was overall not significantly different from TE. Read More

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http://dx.doi.org/10.1016/j.ultrasmedbio.2020.03.025DOI Listing

Clinical features and MRI progression of small duct primary sclerosing cholangitis (PSC).

Eur J Radiol 2020 Jun 1;129:109101. Epub 2020 Jun 1.

Karolinska University Hospital, Division of Radiology, Karolinska Institutet, Stockholm, Sweden.

Purpose: First, to evaluate and describe the clinical and MRI progression of patients with small duct primary sclerosing cholangitis (sdPSC), and second, to look for MRI features associated with disease progression to large duct PSC.

Method: 16 patients (7 female, 9 male; median age 27 years) with diagnosis of sdPSC and available MR imaging were included in this retrospective dual-center study. Liver function tests (LFTs) and imaging was reviewed in consensus by two radiologists at baseline and follow-up, and compared by means of non-parametric tests, with p < 0. Read More

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http://dx.doi.org/10.1016/j.ejrad.2020.109101DOI Listing

In Vivo H MR Spectroscopy of Biliary Components of Human Gallbladder at 7T.

J Magn Reson Imaging 2020 Jun 5:e27207. Epub 2020 Jun 5.

High-field MR Centre, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria.

Background: Previous in vivo proton MR spectroscopy (MRS) studies have demonstrated the possibility of quantifying amide groups of conjugated bile acids (NHCBA), olefinic lipids and cholesterol (OLC), choline-containing phospholipids (CCPLs), taurine and glycine conjugated bile acids (TCBA, GCBA), methylene group of lipids (ML), and methyl groups of bile acids, lipids, and cholesterol (BALC1.0, BALC0.9, and TBAC) in the gallbladder, which may be useful for the study of cholestatic diseases and cholangiopathies. Read More

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http://dx.doi.org/10.1002/jmri.27207DOI Listing

Greater Peripouch Fat Area on CT Image Is Associated with Chronic Pouchitis and Pouch Failure in Inflammatory Bowel Diseases Patients.

Dig Dis Sci 2020 Jun 4. Epub 2020 Jun 4.

Department of Gastroenterology/Hepatology, The Cleveland Clinic Foundation, Cleveland, OH, USA.

Background: The causes of chronic antibiotic refractory pouchitis (CARP) and pouch failure in inflammatory bowel disease (IBD) patients remain unknown. Our previous small study showed peripouch fat area measured by MRI was associated with pouchitis.

Aims: To explore the relationship between peripouch fat area on CT imaging and pouch outcomes. Read More

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http://dx.doi.org/10.1007/s10620-020-06363-7DOI Listing

[Surgical treatment of primary sclerosing cholangitis : Experiences from 30 years in a single center cohort with 173 consecutive patients].

Chirurg 2020 Jun 2. Epub 2020 Jun 2.

Klinik und Poliklinik für Allgemein‑, Viszeral‑, Thorax- und Gefäßchirurgie, Universitätsklinikum Bonn, Venusberg-Campus 1, 53127, Bonn, Deutschland.

Background: In recent years substantial progress has been made in the treatment, surveillance and understanding of the pathogenesis of primary sclerosing cholangitis (PSC); however, in most cases liver transplantation (LTX) is still the only curative option for cancer or end-stage liver disease (ELD). In rare cases a partial liver resection is a possible curative treatment of a PSC-associated cholangiocellular carcinoma (CCC). These operations represent a significant additional burden for PSC patients. Read More

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http://dx.doi.org/10.1007/s00104-020-01197-5DOI Listing

Fenofibrate improves liver function and reduces the toxicity of the bile acid pool in patients with primary biliary cholangitis and primary sclerosing cholangitis who are partial responders to Ursodiol.

Clin Pharmacol Ther 2020 Jun 1. Epub 2020 Jun 1.

Yale University School of Medicine, Liver Center, New Haven, CT, USA.

Cholestatic liver diseases result in the hepatic retention of bile acids, causing subsequent liver toxicity. Peroxisome proliferator-activated receptor alpha (PPARα) regulates bile acid metabolism. In this retrospective observational study, we assessed the effects of fenofibrate (a PPARα agonist) therapy on bile acid metabolism when given to patients with PBC and PSC who have had an incomplete response to Ursodiol monotherapy. Read More

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http://dx.doi.org/10.1002/cpt.1930DOI Listing

The management of gallbladder polyps.

J Visc Surg 2020 May 27. Epub 2020 May 27.

Digestive and transplantation Department, Lille Nord de France University, Claude Huriez Hospital, University Hospital of Lille, rue Michel-Polonovski, 59037 Lille, France.

Neoplastic gallbladder polyps (NGP) are rare; the prevalence in the overall population is less than 10%. NGP are associated with a risk of malignant degeneration and must be distinguished from other benign gallbladder polypoid lesions that occur more frequently. NGP are adenomas and the main risk associated with their management is to fail to detect their progression to gallbladder cancer, which is associated with a particular poor prognosis. Read More

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http://dx.doi.org/10.1016/j.jviscsurg.2020.04.008DOI Listing

The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) index: a prognostic tool for children.

Hepatology 2020 May 28. Epub 2020 May 28.

University of Utah and Intermountain Primary Children's Hospital, Salt Lake City, UT, USA.

Background & Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC. Read More

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http://dx.doi.org/10.1002/hep.31393DOI Listing
May 2020
11.055 Impact Factor

Roles of trained immunity in the pathogenesis of cholangiopathies: a novel therapeutic target.

Hepatology 2020 May 28. Epub 2020 May 28.

Jiangsu Key Laboratory of Immunity and Metabolism, Department of Pathogenic Biology and Immunology, Laboratory of Infection and Immunity, Xuzhou Medical University, Xuzhou, People's Republic of China.

Cholangiopathies are a group of diseases mainly including primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), biliary atresia (BA), and cholangiocarcinoma (CCA). Very limited treatment options are available for these diseases. Following an injury and exposure to DAMPs (Damage associated molecular patterns) and/or PAMPs (Pathogen-associated molecular patterns), cholangiocytes produce a variety of pro-inflammatory cytokines/chemokines leading to the activation of other innate and also adaptive immune cells. Read More

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http://dx.doi.org/10.1002/hep.31395DOI Listing
May 2020
11.055 Impact Factor

Genetic Sequencing of Pediatric Patients Identifies Mutations in Monogenic Inflammatory Bowel Disease Genes that Translate to Distinct Clinical Phenotypes.

Clin Transl Gastroenterol 2020 Feb;11(2):e00129

Department of Human Genetics and Genomic Medicine, University of Southampton, Southampton, UK.

Objectives: Monogenic inflammatory bowel disease (IBD) comprises rare Mendelian causes of gut inflammation, often presenting in infants with severe and atypical disease. This study aimed to identify clinically relevant variants within 68 monogenic IBD genes in an unselected pediatric IBD cohort.

Methods: Whole exome sequencing was performed on patients with pediatric-onset disease. Read More

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http://dx.doi.org/10.14309/ctg.0000000000000129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7145023PMC
February 2020

Hepatocyte KLF6 expression affects FXR signalling and the clinical course of primary sclerosing cholangitis.

Liver Int 2020 May 27. Epub 2020 May 27.

Department of Gastroenterology, Hepatology and Infectious Diseases, Otto-von-Guericke-University Hospital Magdeburg, Magdeburg, Germany.

Background & Aims: 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 are yet available on its potential role in cholestasis. Here, we aimed to identify the impact of hepatic KLF6 expression on cholestatic liver injury and PSC and identify potential effects on farnesoid-X-receptor (FXR) signalling. Read More

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http://dx.doi.org/10.1111/liv.14542DOI Listing

[Recent Updates of Immunoglobulin G4-related Pancreatobiliary Disease].

Korean J Gastroenterol 2020 May;75(5):257-263

Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.

Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis (IgG4-SC) are the pancreatobiliary manifestations of IgG4-related disease. IgG4-related disease is a newly named fibroinflammatory condition that is characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells and often by elevated serum IgG4 concentrations. IgG4-related pancreatobiliary disease is often disguised as pancreatobiliary malignancies owing to its tumefactive nature and clinical presentations, such as obstructive jaundice. Read More

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http://dx.doi.org/10.4166/kjg.2020.75.5.257DOI Listing

[Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis].

Authors:
Dong-Won Ahn

Korean J Gastroenterol 2020 May;75(5):246-256

Department of Internal Medicine, Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea.

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases that ultimately results in the need for liver transplantation in most patients. Imaging studies, such as MRCP, have an essential role in the diagnosis of most cases of PSC. Read More

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http://dx.doi.org/10.4166/kjg.2020.75.5.246DOI Listing

Colonoscopy Surveillance in Liver Transplant Recipients for Primary Sclerosing Cholangitis Associated With Inflammatory Bowel Disease.

Transplant Proc 2020 May 20. Epub 2020 May 20.

Department of General and Transplantation Surgery, Medical University of Warsaw, Warsaw, Poland. Electronic address:

Objective: The aim of this study was to assess gastrointestinal (GI) monitoring in the group of patients who underwent orthotopic liver transplantation (OLTx) because of primary sclerosing cholangitis (PSC) concomitant with inflammatory bowel disease (IBD).

Methods: Analysis was performed of data collected from medical histories and telephone calls in 33 patients who underwent OLTx in the Department of General and Transplantation Surgery of the Medical University of Warsaw from 2001 through 2017 because of PSC concomitant with IBD.

Results: Only 52% of patients claimed they stayed under constant supervision of a GI clinic. Read More

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http://dx.doi.org/10.1016/j.transproceed.2020.01.116DOI Listing

Effects of Primary Sclerosing Cholangitis on Risks of Cancer and Death in People With Inflammatory Bowel Diseases, Based on Sex, Race, and Age.

Gastroenterology 2020 May 20. Epub 2020 May 20.

NIHR Birmingham Biomedical Research Centre, Centre for Liver and Gastroenterology Research, University of Birmingham, UK; Institute of Immunology and Immunotherapy, University of Birmingham, UK; Toronto Centre for Liver Disease, University Health Network and Department of Medicine, University of Toronto, Toronto, Canada. Electronic address:

Background & Aims: There are insufficient population-level data on effects of primary sclerosing cholangitis (PSC) in patients with inflammatory bowel disease (IBD).

Methods: We identified incident cases of IBD, with PSC (PSC-IBD) and without, from April 2006 through to April 2016 and collected data on outcomes through April 2019. We linked data from national healthcare registries maintained for all adults in England on hospital attendances, imaging and endoscopic evaluations, surgical procedures, cancer, and deaths. Read More

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http://dx.doi.org/10.1053/j.gastro.2020.05.049DOI Listing

Risk factors for recurrent autoimmune liver diseases after liver transplantation: A meta-analysis.

Medicine (Baltimore) 2020 May;99(20):e20205

Department of Hepatobiliary Surgery, Dongfang Hospital, Xiamen University.

Background: Autoimmune liver disease (ALD) is a chronic liver disease caused by immune dysfunction in the body. However, no causative or curative medical treatment with proven efficacy exists to cure ALDs, and liver transplantation (LT) remains the only effective treatment available. However, the problem of recurrence of ALDs (rALDs) still remains after LT, which seriously affects the survival rate of the patients. Read More

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http://dx.doi.org/10.1097/MD.0000000000020205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253929PMC

Pediatric Cholestatic Liver Disease: Review of Bile Acid Metabolism and Discussion of Current and Emerging Therapies.

Front Med (Lausanne) 2020 5;7:149. Epub 2020 May 5.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States.

Cholestatic liver diseases are a significant cause of morbidity and mortality and the leading indication for pediatric liver transplant. These include diseases such as biliary atresia, Alagille syndrome, progressive intrahepatic cholestasis entities, ductal plate abnormalities including Caroli syndrome and congenital hepatic fibrosis, primary sclerosing cholangitis, bile acid synthesis defects, and certain metabolic disease. Medical management of these patients typically includes supportive care for complications of chronic cholestasis including malnutrition, pruritus, and portal hypertension. Read More

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http://dx.doi.org/10.3389/fmed.2020.00149DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7214672PMC

Novel CLDN1 Deletion Associated with Ichthyosis, Sclerosing Cholangitis and Acquired Alopecia.

Acta Derm Venereol 2020 Jun 11;100(13):adv00173. Epub 2020 Jun 11.

Department of Dermatology, Necker Hospital, Paris, France.

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http://dx.doi.org/10.2340/00015555-3522DOI Listing

Magnetic resonance imaging features of small-duct primary sclerosing cholangitis.

Abdom Radiol (NY) 2020 May 16. Epub 2020 May 16.

Department of Radiology, Mayo Clinic College of Medicine, Mayo Clinic, 200, First Street SW, Rochester, MN, 55905, USA.

Purpose: To evaluate the biliary tree and hepatic parenchymal findings on magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) in small-duct primary sclerosing cholangitis (SD-PSC).

Methods: Thirty-nine patients with biopsy-proven primary sclerosing cholangitis (PSC) without any bile duct abnormality on MRCP (n = 15) or ERCP (n = 24) at the time of diagnosis were identified. Follow-up MRCP was available in 36/39 patients (12/15 Baseline MRCP group and 24 Baseline ERCP group). Read More

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http://dx.doi.org/10.1007/s00261-020-02572-wDOI Listing

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis.

J Clin Med 2020 May 13;9(5). Epub 2020 May 13.

Liver Center, Autoimmune and Cholestatic Liver Disease Program, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, 110 Francis St. Suite 8E, Boston, MA 02215, USA.

The treatment of overlap syndromes is guided by small observational studies whose data have never been synthesized in a rigorous, quantitative manner. We conducted a systematic review and meta-analysis to evaluate the efficacy of available treatments for these rare and morbid conditions. We searched the literature for studies comparing ≥2 therapies for autoimmune hepatitis (AIH)-primary biliary cholangitis (PBC), AIH-primary sclerosing cholangitis (PSC), PBC-PSC, AIH-PBC-PSC, autoimmune cholangitis (AIC), or autoimmune sclerosing cholangitis (ASC) with respect to various clinical outcomes, including biochemical improvement and transplant-free survival. Read More

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http://dx.doi.org/10.3390/jcm9051449DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7291241PMC

Inflammatory bowel disease and pancreatic cancer: a Scandinavian register-based cohort study 1969-2017.

Aliment Pharmacol Ther 2020 Jul 15;52(1):143-154. Epub 2020 May 15.

Stockholm, Sweden.

Background: Patients with inflammatory bowel disease (IBD) have an increased risk of cancer.

Aim: To assess the risk of pancreatic cancer in IBD compared to the general population.

Methods: Patients with incident IBD 1969-2017 were identified in Danish and Swedish National Patient Registers and through biopsy data, and were matched to IBD-free reference individuals by sex, age, place of residence and year of IBD diagnosis. Read More

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http://dx.doi.org/10.1111/apt.15785DOI Listing

Comparison of liver T1 relaxation times without and with iron correction in pediatric autoimmune liver disease.

Pediatr Radiol 2020 Jun 14;50(7):935-942. Epub 2020 May 14.

Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA.

Background: Magnetic resonance imaging (MRI) T1 relaxometry (mapping) has been reported as a quantitative biomarker of liver injury due to inflammation and fibrosis.

Objective: To assess the relationship between liver MRI T1 relaxometry measurements obtained using a modified Look-Locker inversion recovery (MOLLI) pulse sequence without and with iron (T2*) correction (cT1) in pediatric autoimmune liver disease.

Materials And Methods: This cross-sectional study was institutional review board-approved, with informed consent obtained. Read More

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http://dx.doi.org/10.1007/s00247-020-04663-8DOI Listing
June 2020
1.651 Impact Factor

Prognostic value of peri-nuclear Anti Neutrophil Cytoplasmic Antibodies in subjects with primary sclerosing cholangitis.

Minerva Med 2020 May 14. Epub 2020 May 14.

National Institute of Gastroenterology, "S. de Bellis" Research Hospital Castellana Grotte, Castellana, Bari, Italy.

Objective: To evaluate the prognostic value of peri-nuclear Anti- Neutrophil Cytoplasmic Antibodies (p-ANCA) in subjects with primary sclerosing cholangitis (PSC) and in particular whether it is associated with inflammatory bowel disease (IBD).

Patients And Methods: In the last 9 years,64 patientswere admitted to our Hospital with PSC: 34 males, mean age 53 years, range 17-90. All the patients had a cholestatic pattern of elevated serum enzymes. Read More

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http://dx.doi.org/10.23736/S0026-4806.20.06604-5DOI Listing

The Role of the Intestine in the Pathogenesis of Primary Sclerosing Cholangitis: Evidence and Therapeutic Implications.

Hepatology 2020 May 11. Epub 2020 May 11.

Northwestern University, Department of Internal Medicine, Division of Gastroenterology and Hepatology, Feinberg School of Medicine, 676 N. St. Clair St, 19-045, Chicago, IL, USA.

The pathogenesis of primary sclerosing cholangitis (PSC), a progressive biliary tract disease without approved medical therapy, is not well understood. The relationship between PSC and inflammatory bowel disease (IBD) has inspired theories that intestinal factors that may contribute to the development and progression of hepatobiliary fibrosis in PSC. There is evidence from both fecal and mucosa-associated microbial studies that PSC patients harbor an abnormal enteric microbiome. Read More

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http://dx.doi.org/10.1002/hep.31311DOI Listing