6,503 results match your criteria Cholangitis Primary Sclerosing


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

Eur J Clin Invest 2019 Feb 14:e13088. Epub 2019 Feb 14.

Sheila Sherlock Liver Centre and UCL Institute for Liver and Digestive Health, University College London & Royal Free London NHS Foundation Trust, London, United Kingdom.

Background: 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. Read More

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http://dx.doi.org/10.1111/eci.13088DOI Listing
February 2019

A New Mutation Causing Progressive Familiar Intrahepatic Cholestasis Type 3 in Association with Autoimmune Hepatitis.

Eur J Case Rep Intern Med 2017 27;4(2):000537. Epub 2017 Mar 27.

Liver and Pancreatic Transplantation Unit, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal.

Background: Some patients exhibit features of both autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). Similarly, patients with progressive familial intrahepatic cholestasis type 3 (PFIC3) may share histological features with PSC.

Case Report: We report the case of a 22-year-old man who, since he was 5 years of age, has presented with pruritus, an approximately ninefold elevation of aminotransferases, and γ-glutamyl transferase levels ~10 times the upper limit. Read More

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http://dx.doi.org/10.12890/2016_000537DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346900PMC

Systematic review with meta-analysis: risk factors for recurrent primary sclerosing cholangitis after liver transplantation.

Aliment Pharmacol Ther 2019 Feb 10. Epub 2019 Feb 10.

Department of Gastroenterology and Hepatology, Leiden University Medical Centre, Leiden, The Netherlands.

Background: After liver transplantation primary sclerosing cholangitis (PSC), the condition returns in the transplanted liver in a subset of patients (recurrent primary sclerosing cholangitis, rPSC).

Aim: To define risk factors for rPSC.

Methods: We searched Pubmed, Embase, Web of Science, and Cochrane library for articles published until March 2018. Read More

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http://dx.doi.org/10.1111/apt.15148DOI Listing
February 2019
1 Read

In vivo H MRS of human gallbladder bile in understanding the pathophysiology of primary sclerosing cholangitis (PSC): Immune-mediated disease versus bile acid-induced injury.

NMR Biomed 2019 Feb 8:e4065. Epub 2019 Feb 8.

University of Manitoba, Winnipeg, Manitoba, Canada.

Primary sclerosing cholangitis (PSC) has been considered to be either an "autoimmune disease" or a "bile acid-induced injury." In vitro MRS studies on PSC patients have limitations due to the contamination of bile with contrast agent (commonly administered during endoscopic retrograde cholangiopancreatography) and/or the use of patient cohorts with other diseases as controls. The objective of this study was to quantify biliary metabolites using in vivo H MRS and gain insight into the pathogenesis of PSC. Read More

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http://dx.doi.org/10.1002/nbm.4065DOI Listing
February 2019
1 Read

Fecal Microbiota Transplantation in Patients With Primary Sclerosing Cholangitis: A Pilot Clinical Trial.

Am J Gastroenterol 2019 Jan 31. Epub 2019 Jan 31.

Division of Gastroenterology, Hepatology and Endoscopy, Brigham and Women's Hospital, Boston, Massachusetts, USA.

Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with no effective medical therapies. A perturbation of the gut microbiota has been described in association with PSC, and fecal microbiota transplantation (FMT) has been reported to restore the microbiome in other disease states. Accordingly, we aimed at evaluating the safety, change in liver enzymes, microbiota, and metabolomic profiles in patients with PSC after FMT. Read More

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http://dx.doi.org/10.14309/ajg.0000000000000115DOI Listing
January 2019
1 Read

Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management.

Dig Dis Sci 2019 Feb 6. Epub 2019 Feb 6.

Section of Gastroenterology, Evans Department of Medicine, Boston University School of Medicine, Suite 7717, 85 E Concord St, Boston, MA, 02118, USA.

Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterized by progressive idiopathic stricturing of the biliary system, typically leading to cirrhosis, end-stage liver disease, and colonic or hepatobiliary malignancy. Its presentation is often that of asymptomatic alkaline phosphatase elevation. When symptoms are present, they typically include fatigue, pruritus, or jaundice. Read More

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http://dx.doi.org/10.1007/s10620-019-05484-yDOI Listing
February 2019
1 Read

Amelioration of Ductular Reaction by Stem Cell Derived Extracellular Vesicles in MDR2 knockout mice via let-7 microRNA.

Hepatology 2019 Feb 5. Epub 2019 Feb 5.

Research, Central Texas Veterans Health Care System, Temple, TX, USA.

Cholangiopathies are diseases that affect cholangiocytes, the cells lining the biliary tract. Liver stem cells (LSCs) are able to differentiate into all cells of the liver and possibly influence the surrounding liver tissue by secretion of signaling molecules. One way in which cells can interact is through secretion of extracellular vesicles (EVs), which are small membrane-bound vesicles, containing proteins, miRNAs and cytokines. Read More

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http://dx.doi.org/10.1002/hep.30542DOI Listing
February 2019
1 Read

Severe hypocalcemia following denosumab treatment in a patient with secondary osteoporosis associated with primary sclerosing cholangitis.

Endocr J 2019 Feb 2. Epub 2019 Feb 2.

Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.

Primary sclerosing cholangitis (PSC) has been known as a cause of secondary osteoporosis, which often requires medication. Herein, we give the first report of a case of a 38-year-old man with fatigue and paralysis in both upper limbs who had been treated with denosumab for secondary osteoporosis associated with PSC. Since bisphosphonate (alendronate) was ineffective in our patient, the treatment was changed from alendronate to denosumab. Read More

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http://dx.doi.org/10.1507/endocrj.EJ18-0545DOI Listing
February 2019
1 Read

Treatment of primary sclerosing cholangitis in children.

World J Hepatol 2019 Jan;11(1):19-36

Department of Pediatrics, University of Utah, Salt Lake City, UT 84113, United States.

Primary sclerosing cholangitis (PSC) is a rare disease of stricturing and destruction of the biliary tree with a complex genetic and environmental etiology. Most patients have co-occurring inflammatory bowel disease. Children generally present with uncomplicated disease, but undergo a variable progression to end-stage liver disease. Read More

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http://dx.doi.org/10.4254/wjh.v11.i1.19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354124PMC
January 2019
1 Read

Variety of lung involvement in autoimmune liver diseases.

Ter Arkh 2018 Aug;90(8):107-112

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Faculty of Medicine, Chair of Internal Medicine No.1.

The primary autoimmune liver diseases conventionally include primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis. Despite of primary autoimmune affection of different parts of the hepatobiliary system, in the recent decades, a lot of data has emerged indicating the presence of extrahepatic manifestations of these diseases, in particular, lung lesions, such as nodular and interstitial changes with possible progression and development of fibrosis and respiratory failure. In case of lungs disease, both pulmonary parenchyma and lung vessels, pleura, and intrathoracic lymph nodes can be involved. Read More

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http://dx.doi.org/https://doi.org/10.26442/terarkh2018908107-112DOI Listing
August 2018
1 Read

Disease associated with immunoglobulin g.

Authors:
A G Chuchalin

Ter Arkh 2018 Apr;90(3):4-9

Scientific research Institute pulmonology Federal medical and biological Agency, Moscow, Russia.

Diseases associated with disorders of immunoglobulin g synthesis and its subclasses are described. The analysis of the role of both deficiency and its increased production in the diagnosis and treatment of a number of diseases in humans. Read More

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http://dx.doi.org/10.26442/terarkh20189034-9DOI Listing
April 2018
1 Read

Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.

J Dig Dis 2019 Jan 31. Epub 2019 Jan 31.

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Objective: Autoimmune pancreatitis (AIP) was autoimmune disorder accompanied by clinicopathological manifestation whose concept was established as IgG4-related disease (IgG4-RD). Other IgG4-RDs are often involved with autoimmune pancreatitis, sometimes relapsing despite the favorable response to a steroid therapy. This study aimed to clarify patterns and risk factors for extrapancreatic relapse. Read More

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http://dx.doi.org/10.1111/1751-2980.12708DOI Listing
January 2019
2 Reads

Specific Features of Patients With Inflammatory Bowel Disease and Primary Sclerosing Cholangitis.

J Clin Med Res 2019 Feb 5;11(2):81-88. Epub 2019 Jan 5.

Department of Gastroenterology and Hepatology, Faculty of Medicine, University of Ioannina, Ioannina, Greece.

Primary sclerosing cholangitis (PSC) is a chronic and progressive disease of the biliary tract. PSC is strongly associated with inflammatory bowel disease (IBD), mainly with ulcerative colitis, and most PSC patients have underlying IBD. The pathophysiological interactions between IBD and PSC are unclear, although it seems that the patients with IBD and PSC have a distinct phenotype. Read More

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http://dx.doi.org/10.14740/jocmr3680DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340671PMC
February 2019
1 Read

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

Aliment Pharmacol Ther 2019 Jan 27. Epub 2019 Jan 27.

Department of Internal Medicine IV, University Hospital Heidelberg, Heidelberg, Germany.

Background: 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.

Aim: To perform a longitudinal analysis of CA19-9 in patients with primary sclerosing cholangitis. Read More

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http://dx.doi.org/10.1111/apt.15146DOI Listing
January 2019
1 Read

The Association Between Helicobacter Pylori Infection and Liver and Biliary Tract Disorders.

Curr Health Sci J 2018 Apr-Jun;44(2):186-191. Epub 2018 Mar 27.

University of Oradea, Faculty of Medicine and Pharmacy.

Helicobacter Pylori (HP) persistently colonizes the stomach in about 50% of the globe population and it is the main risk factor for peptic ulcer, as well as for gastric adenocarcinoma and MALT gastric lymphoma. The treatment for HP revolutionized the management of the peptic ulcer disease, providing permanent healing in many cases. Preventing colonization of HP would be the primary prevention of gastric malignancy and peptic ulceration. Read More

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http://dx.doi.org/10.12865/CHSJ.44.02.16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320469PMC
March 2018
9 Reads

[Research advances of autoimmune liver diseases in 2018].

Authors:
Y Chen Q X Wang X Ma

Zhonghua Gan Zang Bing Za Zhi 2019 Jan;27(1):14-17

Department of Gastroenterology, Renji Hospital, Shanghai Jiaotong University School of Medicine;Institute of Digestive Disease, Shanghai 200001, China.

Autoimmune liver disease is a group of hepatobiliary injuries mediated by abnormal immunity. It mainly includes autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis. Recently, an advancement of diagnostic technology has improved the detection and treatment of autoimmune hepatitis. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1007-3418.2019.01.005DOI Listing
January 2019
2 Reads

Combined liver and hematopoietic stem cell transplantation in X-linked hyper IgM syndrome.

J Allergy Clin Immunol 2019 Jan 22. Epub 2019 Jan 22.

Laboratory of Childhood Immunology, Department of Microbiology and Immunology, KU Leuven, Leuven, Belgium, and Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium. Electronic address:

Liver disease in X-linked hyper IgM syndrome (XHIGM) is an important predictor of mortality. In case liver transplantation (LT) is required, a survival benefit is observed when LT is combined with HSCT. Read More

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http://dx.doi.org/10.1016/j.jaci.2018.12.1013DOI Listing
January 2019
3 Reads

Fluocinolone acetonide intravitreal implant as a therapeutic option for severe Sjögren's syndrome-related keratopathy: a case report.

J Med Case Rep 2019 Jan 24;13(1):21. Epub 2019 Jan 24.

Department of Ophthalmology, University Medical Center of the Johannes Gutenberg-University Mainz, Langenbeckstrasse 1, 55131, Mainz, Germany.

Background: In this report, we present the results of a severe case of Sjögren's syndrome-related keratopathy after fluocinolone acetonide 190-μg intravitreal implant (Iluvien®; Alimera Sciences Inc.) therapy.

Case Presentation: A 52-year-old Caucasian woman with Sjögren's syndrome secondary to autoimmune hepatitis and primary sclerosing cholangitis was admitted to our emergency department owing to bilateral corneal ulcers and corneal perforation in the left eye following exposure keratopathy in an artificially induced coma. Read More

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http://dx.doi.org/10.1186/s13256-018-1916-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345050PMC
January 2019
1 Read

M1-Polarized Macrophages Promote Self-Renewing Phenotype of Hepatic Progenitor Cells with Jagged1-Notch Signalling Involved: Relevance in Primary Sclerosing Cholangitis.

J Immunol Res 2018 24;2018:4807145. Epub 2018 Dec 24.

Department of Immunology, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.

The immunologic interaction between parenchyma cells and encircling inflammatory cells is thought to be the most important mechanism of biliary damage and repair in primary sclerosing cholangitis (PSC). Monocytes/macrophages as master regulators of hepatic inflammation have been demonstrated to contribute to PSC pathogenesis. Macrophages coordinate with liver regeneration, and multiple phenotypes have been identified with diverse expressions of surface proteins and cytokine productions. Read More

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http://dx.doi.org/10.1155/2018/4807145DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323443PMC
December 2018
1 Read

[Inflammatory and neoplastic cholangiopathies.]

Recenti Prog Med 2018 Dec;109(12):595-599

Dipartimento di Medicina Interna e Specialità Mediche, Policlinico Umberto I, Sapienza Università di Roma.

The biliary tree consists of mature epithelial cells called cholangiocytes and is subdivided in intra and extrahepatic bile ducts. They facilitate the secretion and modification of the bile constituents and act as transport ducts of bile to the intestine. The alteration of the normal function of cholangiocyte, can lead to the development of multiple biliary diseases, known as cholangiopathies, generally chronic, with a progressive course and which often are lacking of an effective treatment, determining a poor prognosis, even lethal, for the patient. Read More

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http://www.recentiprogressi.it/articoli.php?archivio=yes&
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http://dx.doi.org/10.1701/3082.30744DOI Listing
December 2018
5 Reads

A pilot study of fecal bile acid and microbiota profiles in inflammatory bowel disease and primary sclerosing cholangitis.

Clin Exp Gastroenterol 2019 10;12:9-19. Epub 2019 Jan 10.

Inflammatory Bowel Program, Division of Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, MN, USA,

Introduction: Inflammatory bowel disease (IBD) is thought to arise from an abnormal immune response to the gut microbiota. IBD is associated with altered intestinal microbial community structure and functionality, which may contribute to inflammation and complications such as colon cancer and liver disease. Primary sclerosing cholangitis (PSC) is associated with IBD and markedly increases the risk of colon cancer. Read More

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https://www.dovepress.com/a-pilot-study-of-fecal-bile-acid-a
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http://dx.doi.org/10.2147/CEG.S186097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330977PMC
January 2019
4 Reads

The nonsteroidal FXR agonist cilofexor (GS-9674) improves markers of cholestasis and liver injury in patients with PSC.

Hepatology 2019 Jan 19. Epub 2019 Jan 19.

Liver Care Network and Organ Care Research, Swedish Medical Center, WA, USA.

Primary sclerosing cholangitis (PSC) represents a major unmet medical need. In a Phase 2 double-blind, placebo-controlled study, we tested the safety and efficacy of cilofexor (formerly GS-9674), a nonsteroidal farnesoid X receptor (FXR) agonist in non-cirrhotic patients with large duct PSC. Patients were randomized to receive cilofexor 100 mg (n=22), 30 mg (n=20), or placebo (n=10) orally once daily for 12 weeks. Read More

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http://doi.wiley.com/10.1002/hep.30509
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http://dx.doi.org/10.1002/hep.30509DOI Listing
January 2019
14 Reads

Hematopoietic stem cell transplantation for CD40 ligand deficiency: results from an EBMT/ESID-IEWP-SCETIDE-PIDTC Study.

J Allergy Clin Immunol 2019 Jan 17. Epub 2019 Jan 17.

Department of Pediatric Immunology and HSCT, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK; Institute of Cellular Medicine, Newcastle University, Framlington Place, Newcastle upon Tyne - UK, NE2 4HH.

Background: CD40 ligand (CD40L) deficiency, an X-linked primary immunodeficiency, causes recurrent sinopulmonary, Pneumocystis and Cryptosporidium infections. Long-term survival with supportive therapy is poor. Currently, the only curative treatment is hematopoietic stem cell transplantation (HSCT). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193003
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http://dx.doi.org/10.1016/j.jaci.2018.12.1010DOI Listing
January 2019
6 Reads

Better end points needed in primary sclerosing cholangitis trials.

Nat Rev Gastroenterol Hepatol 2019 Jan 17. Epub 2019 Jan 17.

Division of Gastroenterology and Center for Autoimmune Liver Diseases, San Gerardo Hospital, Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.

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http://dx.doi.org/10.1038/s41575-019-0110-5DOI Listing
January 2019
2 Reads

Serum levels of miR-29, miR-122, miR-155 and miR-192 are elevated in patients with cholangiocarcinoma.

PLoS One 2019 17;14(1):e0210944. Epub 2019 Jan 17.

Department of Medicine III, University Hospital RWTH Aachen, Aachen, Germany.

Objectives: Cholangiocarcinoma (CCA) represents the second most common primary hepatic malignancy. Despite tremendous research activities, the prognosis for the majority of patients is still poor. Only in case of early diagnosis, liver resection might potentially lead to long-term survival. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210944PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6336320PMC
January 2019
1 Read

Adult liver transplantation in Johannesburg, South Africa (2004 - 2016): Balancing good outcomes, constrained resources and limited donors.

S Afr Med J 2018 Oct 26;108(11):929-936. Epub 2018 Oct 26.

Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa; Department of Internal Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.

Background: Liver transplantation is the standard of care for the treatment of liver failure worldwide, yet millions of people living in sub-Saharan Africa remain without access to these services. South Africa (SA) has two liver transplant centres, one in Cape Town and the other in Johannesburg, where Wits Donald Gordon Medical Centre (WDGMC) started an adult liver transplant programme in 2004.

Objectives:  To describe the outcomes of the adult liver transplant programme at WDGMC. Read More

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http://www.samj.org.za/index.php/samj/article/view/12463
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http://dx.doi.org/10.7196/SAMJ.2018.v108i11.13286DOI Listing
October 2018
6 Reads

Gut pathobionts underlie intestinal barrier dysfunction and liver T helper 17 cell immune response in primary sclerosing cholangitis.

Nat Microbiol 2019 Jan 14. Epub 2019 Jan 14.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Shinanomachi, Tokyo, Japan.

Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease and its frequent complication with ulcerative colitis highlights the pathogenic role of epithelial barrier dysfunction. Intestinal barrier dysfunction has been implicated in the pathogenesis of PSC, yet its underlying mechanism remains unknown. Here, we identify Klebsiella pneumonia in the microbiota of patients with PSC and demonstrate that K. Read More

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http://dx.doi.org/10.1038/s41564-018-0333-1DOI Listing
January 2019
6 Reads

Th17 cell frequency is associated with low bone mass in primary sclerosing cholangitis.

J Hepatol 2019 Jan 11. Epub 2019 Jan 11.

Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany; Martin Zeitz Center for Rare Diseases, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany; University Medical Center Hamburg-Eppendorf, Germany. Electronic address:

Background And Aims: 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.

Methods: We determined bone mineral density by dual-X-ray absorptiometry and assessed bone microstructure by high-resolution peripheral quantitative computed tomography in PSC patients. Read More

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http://dx.doi.org/10.1016/j.jhep.2018.12.035DOI Listing
January 2019
1 Read

Healthy Donor Fecal Microbiota Transplantation for Recurrent Bacterial Cholangitis in Primary Sclerosing Cholangitis - A Single Case Report.

J Clin Transl Hepatol 2018 Dec 1;6(4):438-441. Epub 2018 Aug 1.

Molecular, Cellular and Developmental Biology, Genepath Dx, Phadke Hospital, Shivajinagar, Pune, Maharashtra, India.

Recurrent acute bacterial cholangitis is a unique indication for liver transplantation in primary sclerosing cholangitis. We present the first report on utility of healthy donor fecal transplantation for management of recurrent acute bacterial cholangitis in a primary sclerosing cholangitis patient. We demonstrate the striking liver biochemistry, bile acid and bacterial community changes following intestinal microbiota transplantation associated with amelioration of recurrent cholangitis. Read More

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http://dx.doi.org/10.14218/JCTH.2018.00033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328734PMC
December 2018
3 Reads

Nivolumab-related Sclerosing Cholangitis in Gastric Cancer.

Authors:
Yoshiharu Motoo

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Medical Oncology, Kanazawa Medical University, Japan.

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https://www.jstage.jst.go.jp/article/internalmedicine/advpub
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http://dx.doi.org/10.2169/internalmedicine.2135-18DOI Listing
January 2019
1 Read
0.967 Impact Factor

Efficacy and Safety of Chemotherapy Following Anti-PD-1 Antibody Therapy for Gastric Cancer: A Case of Sclerosing Cholangitis.

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan.

Immunotherapy targeting programmed cell death-1 (PD-1) signaling is becoming the standard of care for advanced gastric cancer. We herein report a patient with gastric adenocarcinoma with peritoneal dissemination who was treated with nab-paclitaxel and ramucirumab following nivolumab and developed sclerosing cholangitis. Endoscopic retrograde cholangiography showed irregular narrowing and widening of the entire intrahepatic biliary system. Read More

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http://dx.doi.org/10.2169/internalmedicine.1981-18DOI Listing
January 2019
1 Read

NGM282, an FGF19 analogue, in primary sclerosing cholangitis: A nebulous matter.

J Hepatol 2019 Jan 6. Epub 2019 Jan 6.

Professor of Medicine and Senior Advisor to the Provost, College of Health Solutions, Arizona State University, Phoenix, AZ, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jhep.2018.12.006DOI Listing
January 2019

Detection of Autoantibodies Against Nucleoporin p62 in Sera of Patients With Primary Biliary Cholangitis.

Ann Lab Med 2019 May;39(3):291-298

Department of Gastroenterology, Hepatology and Clinical Oncology, Centre of Postgraduate Medical Education, Warsaw, Poland.

Background: Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by specific autoantibodies. We evaluated the prevalence of autoantibodies against nucleoporin p62 (anti-p62) in PBC patients' sera to determine whether it can be a marker for PBC, in comparison with other immunological and biochemical parameters. We validated the performance of our in-house ELISA technique. Read More

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https://synapse.koreamed.org/DOIx.php?id=10.3343/alm.2019.39
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http://dx.doi.org/10.3343/alm.2019.39.3.291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340841PMC
May 2019
5 Reads

Current therapies and novel approaches for biliary diseases.

World J Gastrointest Pathophysiol 2019 Jan;10(1):1-10

Department of Medicine, The University of Melbourne, Melbourne, VIC 3084, Australia.

Chronic liver diseases that inevitably lead to hepatic fibrosis, cirrhosis and/or hepatocellular carcinoma have become a major cause of illness and death worldwide. Among them, cholangiopathies or cholestatic liver diseases comprise a large group of conditions in which injury is primarily focused on the biliary system. These include congenital diseases (such as biliary atresia and cystic fibrosis), acquired diseases (such as primary sclerosing cholangitis and primary biliary cirrhosis), and those that arise from secondary damage to the biliary tree from obstruction, cholangitis or ischaemia. Read More

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https://www.wjgnet.com/2150-5330/full/v10/i1/1.htm
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http://dx.doi.org/10.4291/wjgp.v10.i1.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6318481PMC
January 2019
3 Reads

[The gut microbiome is important in chronic liver disease].

Authors:
Erik Christensen

Ugeskr Laeger 2019 Jan;181(2)

There is increasing evidence of gut dysbiosis being a significant factor in the development and progression of important chronic liver diseases including non-alcoholic steatohepatitis (NASH), alcoholic liver disease, primary sclerosing cholangitis and cirrhosis. Dysbiotic microbiota and their toxic metabolites translocate across the gut epithelium via the portal vein and cause damage to the liver. In NASH, alcohol-producing gut bacteria may be critical for its development. Read More

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January 2019
1 Read

Rate of Spleen Length Progression is a Marker of Outcome in Patients with Primary Sclerosing Cholangitis.

Clin Gastroenterol Hepatol 2019 Jan 4. Epub 2019 Jan 4.

Department of Interdisciplinary Endoscopy, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.

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http://dx.doi.org/10.1016/j.cgh.2018.12.033DOI Listing
January 2019
8 Reads

Comprehensive Review of Molecular Mechanisms during Cholestatic Liver Injury and Cholangiocarcinoma.

J Liver 2018 20;7(3). Epub 2018 Sep 20.

Research, Central Texas Veterans Health Care System, Texas, USA.

Cholestatic liver injury is characterized by damage induced on the biliary tree and cholangiocytes, the cells lining the biliary tree, thus they are termed "cholangiopathies". Cholangiopathies include diseases such as Primary Biliary Cholangitis, Primary Sclerosing Cholangitis, Biliary Atresia and Cholangiocarcinoma. These pathologies lack viable therapies and most patients are diagnosed during late stage disease progression (with the exception of Biliary Atresia, which is found shortly after birth). Read More

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http://dx.doi.org/10.4172/2167-0889.1000231DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319937PMC
September 2018
1 Read

Cholangiocyte death in ductopenic cholestatic cholangiopathies: Mechanistic basis and emerging therapeutic strategies.

Life Sci 2019 Feb 2;218:324-339. Epub 2019 Jan 2.

Instituto de Fisiología Experimental (IFISE-CONICET), Facultad de Ciencias Bioquímicas y Farmacéuticas, Universidad Nacional de Rosario (UNR), Suipacha 570, 2000 Rosario, Argentina. Electronic address:

Among hepatic diseases, cholestatic ductopenic cholangiopathies are poorly studied, and they are rarely given the importance they deserve, especially considering their high incidence in clinical practice. Although cholestatic ductopenic cholangiopathies have different etiologies and pathogenesis, all have the same target (the cholangiocyte) and similar mechanistic basis of cell death. Cholestatic cholangiopathies are characterized, predominantly, by obstructive or functional damage in the biliary epithelium, resulting in an imbalance between proliferation and cholangiocellular death; this leads to the progressive disappearance of bile ducts, as has been shown to occur in primary sclerosing cholangitis, primary biliary cholangitis, low-phospholipid-associated cholelithiasis syndrome, cystic fibrosis-related liver disease, and drug-induced ductopenia, among other biliary disorders. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00243205183083
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http://dx.doi.org/10.1016/j.lfs.2018.12.044DOI Listing
February 2019
8 Reads

Loss of Wnt Secretion by Macrophages Promotes Hepatobiliary Injury after Administration of 3,5-Diethoxycarbonyl-1, 4-Dihydrocollidine Diet.

Am J Pathol 2019 Jan 2. Epub 2019 Jan 2.

Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania; Pittsburgh Liver Research Center, University of Pittsburgh and University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. Electronic address:

Exposure of mice to a diet containing 3,5-diethoxycarbonyl-1, 4-dihydrocollidine (DDC) induces porphyrin accumulation, cholestasis, immune response, and hepatobiliary damage mimicking hepatic porphyria and sclerosing cholangitis. Although β-catenin signaling promotes hepatocyte proliferation, and macrophages are a source of Wnts, the role of macrophage-derived Wnts in modulating hepatobiliary injury/repair remains unresolved. We investigated the effect of macrophage-specific deletion of Wntless, a cargo protein critical for cellular Wnt secretion, by feeding macrophage-Wntless-knockout (Mac-KO) and wild-type littermates a DDC diet for 14 days. Read More

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http://dx.doi.org/10.1016/j.ajpath.2018.11.010DOI Listing
January 2019
2 Reads

Increasing Incidence of Nonalcoholic Steatohepatitis as an Indication for Liver Transplantation in Australia and New Zealand.

Liver Transpl 2019 Jan;25(1):25-34

Medical School, University of Western Australia, Nedlands, Australia.

The worldwide increase in obesity and diabetes has led to predictions that nonalcoholic steatohepatitis (NASH) will become the leading indication for orthotopic liver transplantation (OLT). Data supporting this prediction from outside the United States are limited. Thus, we aimed to determine trends in the frequency of NASH among adults listed and undergoing OLT in Australia and New Zealand (ANZ) from 1994 to 2017. Read More

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http://doi.wiley.com/10.1002/lt.25361
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http://dx.doi.org/10.1002/lt.25361DOI Listing
January 2019
5 Reads

Diagnostic performance of quantitative magnetic resonance imaging biomarkers for predicting portal hypertension in children and young adults with autoimmune liver disease.

Pediatr Radiol 2019 Jan 3. Epub 2019 Jan 3.

Center for Autoimmune Liver Disease (CALD), Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Background: Primary sclerosing cholangitis, autoimmune hepatitis and autoimmune sclerosing cholangitis are forms of chronic, progressive autoimmune liver disease (AILD) that can affect the pediatric population.

Objective: To determine whether quantitative MRI- and laboratory-based biomarkers are associated with conventional imaging findings of portal hypertension (radiologic portal hypertension) in children and young adults with AILD.

Materials And Methods: Forty-four patients with AILD enrolled in an institutional registry underwent a research abdominal MRI examination at 1. Read More

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http://link.springer.com/10.1007/s00247-018-4319-1
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http://dx.doi.org/10.1007/s00247-018-4319-1DOI Listing
January 2019
6 Reads
1.651 Impact Factor

Beaded bile ducts in primary sclerosing cholangitis.

Abdom Radiol (NY) 2019 Jan 1. Epub 2019 Jan 1.

Section of Radiological Sciences, Di.Bi.Med, University of Palermo, Via del Vespro 127, 90127, Palermo, Italy.

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http://dx.doi.org/10.1007/s00261-018-1873-9DOI Listing
January 2019
1 Read

GPR35 promotes glycolysis, proliferation, and oncogenic signaling by engaging with the sodium potassium pump.

Sci Signal 2019 Jan 1;12(562). Epub 2019 Jan 1.

Division of Gastroenterology and Hepatology, Department of Medicine, University of Cambridge, Cambridge CB2 0QQ, UK.

The sodium potassium pump (Na/K-ATPase) ensures the electrochemical gradient of a cell through an energy-dependent process that consumes about one-third of regenerated ATP. We report that the G protein-coupled receptor GPR35 interacted with the α chain of Na/K-ATPase and promotes its ion transport and Src signaling activity in a ligand-independent manner. Deletion of Gpr35 increased baseline Ca to maximal levels and reduced Src activation and overall metabolic activity in macrophages and intestinal epithelial cells (IECs). Read More

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http://dx.doi.org/10.1126/scisignal.aau9048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364804PMC
January 2019
5 Reads

Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity.

Ann Hepatol 2018 Dec;18(1):215-219

Liver Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. Read More

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http://dx.doi.org/10.5604/01.3001.0012.7916DOI Listing
December 2018
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Acoustic Radiation Force Impulse (ARFI) Elastography in Autoimmune and Cholestatic Liver Diseases.

Ann Hepatol 2018 Dec;18(1):23-29

Department of Internal Medicine 1, Friedrich-Alexander University Erlangen-Nurnberg, Erlangen, Germany.

Introduction: Acoustic Radiation Force Impulse (ARFI) elastography evaluates hepatic fibrosis non-invasively and has been mainly validated in viral hepatitis. Data on rare liver diseases such as autoimmune hepatitis (AIH), overlap syndrome, primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC) are sparse.

Material And Methods: 85 patients (including 31 AIH, 26 PBC, 16 PSC and 3 PSC- and 9 PBC- AIH-overlap syndromes) were retrospectively analysed pointing at ARFI elastography of the liver and the correlation with histologic Ishak fibrosis score (F0-6). Read More

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http://dx.doi.org/10.5604/01.3001.0012.7858DOI Listing
December 2018
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Ectopic relapse of IgG4-related disease presenting as IgG4-related sclerosing cholecystitis: A case report and review of literature.

Medicine (Baltimore) 2018 Dec;97(52):e13868

Department of Gastroenterology and Hepatology.

Rationale: Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a chronic inflammatory disorder characterized by high levels of serum IgG4, swollen organs with fibrosis and abundant infiltration of IgG4-positive plasmacytes.

Patient Concerns: An 82-year-old male visited our hospital for an evaluation of a pancreatic enlargement and a bilateral submandibular adenopathy. Further investigation revealed elevation of serum IgG4 and bilateral lacrimal submandibular adenopathy. Read More

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http://dx.doi.org/10.1097/MD.0000000000013868DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314772PMC
December 2018
13 Reads

Editorial: deciding when to intervene in primary sclerosing cholangitis.

Aliment Pharmacol Ther 2018 Aug;48(4):484-485

Centre for Liver and Digestive Disorders, Royal Infirmary of Edinburgh, Edinburgh, UK.

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http://dx.doi.org/10.1111/apt.14898DOI Listing
August 2018
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High-throughput sequencing identifies aetiology-dependent differences in ductular reaction in human chronic liver disease.

J Pathol 2018 Dec 25. Epub 2018 Dec 25.

Department of Imaging and Pathology, KU Leuven and University Hospitals Leuven, Leuven, Belgium.

Ductular reaction (DR) represents the activation of hepatic progenitor cells (HPCs) and has been associated with features of advanced chronic liver disease; yet it is not clear whether these cells contribute to disease progression and how the composition of their micro-environment differs depending on the aetiology. This study aimed to identify HPC-associated signalling pathways relevant in different chronic liver diseases using a high-throughput sequencing approach. DR/HPCs were isolated using laser microdissection from patient samples diagnosed with HCV or primary sclerosing cholangitis (PSC), as models for hepatocellular or biliary regeneration. Read More

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http://dx.doi.org/10.1002/path.5228DOI Listing
December 2018
2 Reads