6,557 results match your criteria Primary Sclerosing Cholangitis


Preclinical insights into cholangiopathies: disease modeling and emerging therapeutic targets.

Expert Opin Ther Targets 2019 Apr 16. Epub 2019 Apr 16.

a Indiana Center for Liver Research, Division of Gastroenterology & Hepatology, Department of Medicine , Indiana University School of Medicine , Indianapolis , Indiana , USA.

Introduction The common predominant clinical features of cholangiopathies such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), and biliary atresia (BA) are biliary damage/senescence and liver fibrosis. Curative therapies are lacking, and liver transplantation is only option. An understanding of the mechanisms and pathogenesis is needed to develop novel therapies. Read More

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http://dx.doi.org/10.1080/14728222.2019.1608950DOI Listing
April 2019
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Cholangiocyte-derived exosomal LncRNA H19 promotes hepatic stellate cell activation and cholestatic liver fibrosis.

Hepatology 2019 Apr 15. Epub 2019 Apr 15.

Department of Microbiology and Immunology and McGuire Veterans Affairs Medical Center, Virginia Commonwealth University, Richmond, Virginia, USA.

Activation of hepatic stellate cells (HSCs) represents the primary driving force to promote the progression of chronic cholestatic liver diseases. We previously reported that cholangiocyte-derived exosomal-long non-coding RNA-H19 (LncRNA-H19) plays a critical role in promoting cholestatic liver injury. However, it remains unclear whether cholangiocyte-derived lncRNA-H19 regulates HSC activation, which is the major focus of this study. Read More

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http://dx.doi.org/10.1002/hep.30662DOI Listing

The Role of Next Generation Sequencing in the Differential Diagnosis of Caroli's Syndrome.

Balkan J Med Genet 2018 Dec 31;21(2):49-53. Epub 2018 Dec 31.

Clinical Institute of Medical Genetics, University Medical Centre Ljubljana, Ljubljana, Slovenia.

We report the case of a 41-year-old man on conservative treatment for more than 20 years for chronic renal insufficiency, chronic hepatitis and recurrent cholangitis. Following lengthy and extensive diagnostics, the differential diagnosis included primary sclerosing cholangitis and Caroli's disease (CD). To solve the diagnostic challenge, next generation sequencing (NGS) was performed to distinguish between the disorders possibly present in the patient. Read More

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http://dx.doi.org/10.2478/bjmg-2018-0027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454240PMC
December 2018
1 Read

[Diagnosis of extra pancreatic lesions (focused on IgG4-related sclerosing cholangitis)].

Nihon Shokakibyo Gakkai Zasshi 2019 ;116(4):296-304

Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences.

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http://dx.doi.org/10.11405/nisshoshi.116.296DOI Listing
January 2019
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Integrating next-generation sequencing to endoscopic retrograde cholangiopancreatography (ERCP)-obtained biliary specimens improves the detection and management of patients with malignant bile duct strictures.

Gut 2019 Apr 10. Epub 2019 Apr 10.

Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Objective: Despite improvements in imaging, serum CA19-9 and pathological evaluation, differentiating between benign and malignant bile duct strictures remains a diagnostic conundrum. Recent developments in next-generation sequencing (NGS) have opened new opportunities for early detection and management of cancers but, to date, have not been rigorously applied to biliary specimens.

Design: We prospectively evaluated a 28-gene NGS panel (BiliSeq) using endoscopic retrograde cholangiopancreatography-obtained biliary specimens from patients with bile duct strictures. Read More

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http://dx.doi.org/10.1136/gutjnl-2018-317817DOI Listing
April 2019
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Emerging novel treatments for autoimmune liver diseases.

Authors:
Atsushi Tanaka

Hepatol Res 2019 Apr 10. Epub 2019 Apr 10.

School of Medicine, Teikyo University, Tokyo, Japan.

The etiology of autoimmune liver diseases, such as autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC), still remains largely unknown and no therapeutic agents that are able to "cure" the disease have been developed. While corticosteroids for AIH and ursodeoxycholic acid (UDCA) for PBC have been shown to significantly improve liver transplantation (LT)-free survival and are recommended as first-line drugs, treatment strategies for patients who exhibit incomplete response to these drugs have not yet been fully established. No drug is significantly associated with long LT-free survival in PSC patients. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/hepr.13347
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http://dx.doi.org/10.1111/hepr.13347DOI Listing
April 2019
3 Reads

Duck circovirus induces a new pathogenetic characteristic, primary sclerosing cholangitis.

Comp Immunol Microbiol Infect Dis 2019 Apr 3;63:31-36. Epub 2019 Jan 3.

College of Veterinary Medicine, Shandong Agricultural University, Tai'an, 271018, PR China. Electronic address:

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease of unknown cause. In the study, we found that duck circovirus (DuCV) induces PSC in natural and reproductive cases. PSC in DuCV naturally infected ducks was investigated by PCR and histopathology. Read More

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http://dx.doi.org/10.1016/j.cimid.2018.12.009DOI Listing
April 2019
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Clinical course and mortality by etiology of liver cirrhosis in Sweden: a population based, long-term follow-up study of 1317 patients.

Aliment Pharmacol Ther 2019 Apr 8. Epub 2019 Apr 8.

Department of Clinical Sciences, Lund University, Lund, Sweden.

Background: Liver cirrhosis is characterized by a silent phase until decompensation, which is defined by ascites, bleeding from esophageal varices or hepatic encephalopathy.

Aim: We compare the clinical course, patterns of survival and causes of death by etiology during long-term follow-up in a large population-based cohort of patients with incident cirrhosis.

Material And Methods: We used population-based medical registries for a cohort study of patients with liver cirrhosis diagnosed January 2001 to December 2010, in the Scania region of Sweden. Read More

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http://doi.wiley.com/10.1111/apt.15255
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http://dx.doi.org/10.1111/apt.15255DOI Listing
April 2019
8 Reads

Secondary sclerosing cholangitis in critically ill patients.

Cir Cir 2019 ;86(1):49-55

Medical Management Office. Hospital General Naval de Alta Especialidad, Secretaría de Marina/Armada de México, Mexico City. Mexico.

Primary sclerosing cholangitis (PSC) is a rare idiopathic condition with immunopathogenic mechanisms where there is chronic progressive destruction of the biliary tree. Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, but is caused by specific processes which directly damage the biliary tree; examples include recurrent pancreatitis, bile duct malignancy, congenital bile duct abnormalities. A new cause of SSC has been described during or following significant critical illness associated with severe respiratory insufficiency, vasopressor requirement, shock and sepsis. Read More

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http://dx.doi.org/10.24875/CIRUE.M18000008DOI Listing
January 2019
3 Reads

Primary sclerosing cholangitis and inflammatory bowel disease: Intestine-liver interrelation.

Gastroenterol Hepatol 2019 Apr 1. Epub 2019 Apr 1.

Servicio de Aparato Digestivo, Hospital Clínico Universitario Lozano Blesa, IIS Aragón, Ciberehd, Zaragoza, España.

The association between inflammatory bowel disease (IBD) and primary sclerosing cholangitis should be considered a distinct clinical entity. This association involves genetic abnormalities, epidemiological factors (more common in men, with no a geographical pattern) and, commonly, subclinical inflammation, predominance of the right colon (endoscopic and histological), backwash ileitis and rectal sparing. Furthermore, there is an increased risk of colorectal cancer and cholangiocarcinoma. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02105705193005
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http://dx.doi.org/10.1016/j.gastrohep.2019.02.004DOI Listing
April 2019
2 Reads

Rheumatologic Diseases and the Liver.

Clin Liver Dis 2019 May 8;23(2):247-261. Epub 2019 Mar 8.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Howard University Hospital and College of Medicine, 2041 Georgia Avenue Northwest, Suite 5C02, Washington, DC 20060, USA. Electronic address:

A variety of rheumatologic disorders may affect the liver. There is a significant epidemiologic, genetic, and immunologic overlap between immune-mediated rheumatologic disorders and autoimmune liver diseases. There is an increased frequency of autoimmune liver diseases, such as primary biliary cholangitis, autoimmune hepatitis, primary sclerosing cholangitis, or overlap syndrome, in patients with systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, systemic sclerosis, vasculitis, and other immune-related diseases. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.007DOI Listing
May 2019
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Hepatic Complications of Inflammatory Bowel Disease.

Clin Liver Dis 2019 May 26;23(2):191-208. Epub 2019 Feb 26.

Division of Gastroenterology and Hepatology, University of Miami Miller School of Medicine, 1120 Northwest 14 Street #1115, Miami, FL 33136, USA.

Hepatobiliary disorders are commonly encountered in patients with inflammatory bowel disease (IBD). Although primary sclerosing cholangitis is the stereotypical hepatobiliary disorder associated with IBD, other diseases, including autoimmune hepatitis and nonalcoholic fatty liver disease, also are encountered in this population. Several agents used for treatment of IBD may cause drug-induced liver injury, although severe hepatotoxicity occurs infrequently. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.003DOI Listing
May 2019
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Follicular cholecystitis: Clinicopathologic associations.

Hum Pathol 2019 Apr 1. Epub 2019 Apr 1.

Department of Pathology and Immunology, School of Medicine, Washington University in St. Louis, 425 S Euclid Ave, Campus Box 8118, St. Louis, MO, USA 63110.. Electronic address:

Follicular cholecystitis (FC) is a relatively rare entity with uncertain causal associations. In this study, we aimed to explore different clinicopathologic associations of FC, and to better characterize the entity. A retrospective review of archival H&E slides and pertinent clinical information was undertaken for all cholecystectomy cases with a rendered diagnosis of "follicular cholecystitis", from 1991 to 2017. Read More

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http://dx.doi.org/10.1016/j.humpath.2019.03.003DOI Listing
April 2019
2 Reads

Hepatobiliary and pancreatic manifestations in inflammatory bowel diseases: a referral center study.

BMC Gastroenterol 2019 Apr 3;19(1):48. Epub 2019 Apr 3.

Department of Gastroenterology and Hepatology, Medical school and University of Ioannina, Ioannina, Greece.

Background: Hepatobiliary and pancreatic manifestations have been reported in patients with Crohn's disease or ulcerative colitis. Our aim was to describe the prevalence of hepatobiliary and pancreatic manifestations in inflammatory bowel disease and their association with the disease itself and the medications used.

Methods: Data were retrospectively extracted from the clinical records of patients followed up at our tertiary IBD referral Center. Read More

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http://dx.doi.org/10.1186/s12876-019-0967-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446300PMC
April 2019
2 Reads

An increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan.

Hepatol Res 2019 Apr 1. Epub 2019 Apr 1.

Faculty of Medical Technology, Teikyo University, Tokyo, Japan.

Background And Aim: Autoimmune liver diseases (AILD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) impose a significant burden on public health, and it is important to estimate prevalence of AILD. We conducted a nationwide, hospital-based, epidemiological survey of AIH, PBC, and PSC, and compared the results with those from previous surveys.

Method: We randomly selected healthcare facilities used in the survey from a list of all facilities in Japan. Read More

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http://dx.doi.org/10.1111/hepr.13342DOI Listing
April 2019
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Low-Phospholipid Associated Cholelithiasis (LPAC) syndrome: A synthetic review.

J Visc Surg 2019 Mar 25. Epub 2019 Mar 25.

Service de chirurgie générale, digestive et transplantation hépatique et intestinale (general, digestive, liver transplant and intestinal surgery department), hôpital de la Croix Rousse, hospices civils de Lyon, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France.

Low-Phospholipid Associated Cholelithiasis (LPAC) is a genetic disease responsible for the development of intrahepatic lithiasis. It is associated with a mutation of the ABCB4 gene which codes for protein MDR3, a biliary carrier. As a nosological entity, it is defined by presence of two of the three following criteria: age less than 40 years at onset of biliary symptoms, recurrence of biliary symptoms after cholecystectomy, and intrahepatic hyperechogenic foci detected by ultrasound. Read More

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http://dx.doi.org/10.1016/j.jviscsurg.2019.02.006DOI Listing
March 2019
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Manipulation of the Gut-Liver Axis Using Microbiome Restoration Therapy in Primary Sclerosing Cholangitis.

Am J Gastroenterol 2019 Mar 12. Epub 2019 Mar 12.

Division of Gastroenterology and Hepatology, University of Virginia Health System, Charlottesville, Virginia, USA.

Alteration of the normal gut-liver axis is important in primary sclerosing cholangitis (PSC). Lack of effective medical therapy for PSC makes microbiome restoration an alluring therapeutic target. Allegretti et al. Read More

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http://dx.doi.org/10.14309/ajg.0000000000000191DOI Listing
March 2019
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Difficulty in the diagnosis of isolated IgG4-related sclerosing cholangitis.

Dig Endosc 2019 Mar 28. Epub 2019 Mar 28.

Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, 467-8601, Japan.

Takagi et al. reported that differentiation between isolated IgG4-related sclerosing cholangitis (IgG4-SC showing no association with autoimmune pancreatitis) and Klatskin tumor is difficult because both appear as nodular lesions (1). They concluded that cholangiographic findings with symmetrical smooth long stricture extending into the upper bile duct This article is protected by copyright. Read More

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http://dx.doi.org/10.1111/den.13407DOI Listing
March 2019
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Comparison of Sertraline with Rifampin in the treatment of Cholestatic pruritus: A randomized clinical trial.

Rev Recent Clin Trials 2019 Mar 28. Epub 2019 Mar 28.

Department of Internal Medicine Associate Professor of Gastroenterology and Hepatology, School of Medicine, Hamadan University of Medical Sciences, Hamadan. Iran.

Background: Pruritus is one of the most common and disabling symptoms of liver disease such as Primary Sclerosing Cholangitis and Primary Biliary Cholangitis. Cholestyramine, rifampin, opioid antagonists, antihistaminic agents and SSRIs are used to management of pruritus. Due to rifampin drug interactions as well as its serious side effects such as hepatotoxicity clinicians attempt to find a safer and a more effective substitution. Read More

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http://dx.doi.org/10.2174/1574887114666190328130720DOI Listing
March 2019
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Effect of scheduled endoscopic dilatation of dominant strictures on outcome in patients with primary sclerosing cholangitis.

Gut 2019 Mar 25. Epub 2019 Mar 25.

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

Objective: Scheduled endoscopic dilatation of dominant strictures (DS) in primary sclerosing cholangitis (PSC) might improve outcome relative to endoscopic treatment on demand, but evidence is limited. Since randomisation is difficult in clinical practice, we present a large retrospective study comparing scheduled versus on-demand endoscopic retrograde cholangiopancreatography (ERCP) based on patient preferences.

Design: Between 1987 and 2017, all new patients with PSC had been offered scheduled ERCP with dilatation of a DS if diagnosed; the latter was repeated at defined intervals until morphological resolution, independent of clinical symptoms (treatment group). Read More

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http://dx.doi.org/10.1136/gutjnl-2018-316801DOI Listing
March 2019
1 Read

Bile ductular reactions in the liver: similarities are only skin deep.

J Pathol 2019 Mar 18. Epub 2019 Mar 18.

Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Extensive bile ductular reactions (DRs) accompany many cholestatic liver diseases such as primary biliary cholangitis and primary sclerosing cholangitis (PSC) as well as parenchymal liver cell diseases such as alcoholic liver disease, non-alcoholic steatohepatitis and HCV and HBV infections. DRs originate from bile ducts or hepatocytes after damage and can be identified by expression of markers associated with cholangiocytes, often being associated with disease progression and fibrosis. In a recent issue of The Journal of Pathology, Govaere et al employed high-throughput RNA sequencing to compare the transcriptomic profiles of DR cells from liver diseases of different aetiology; HCV infection affecting hepatocytes and PSC initially affecting biliary epithelial cells. Read More

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http://dx.doi.org/10.1002/path.5265DOI Listing
March 2019
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Intrahepatic diffuse periportal enhancement patterns on hepatobiliary phase gadoxetate disodium-enhanced liver MR images: Do they correspond to periportal hyperintense patterns on T2-weighted images?

Medicine (Baltimore) 2019 Mar;98(11):e14784

Institute for Clinical Radiology, Ludwig Maximilians-University Hospital Munich, Munich, Germany.

The purpose of this study was to investigate the findings of diffuse periportal enhancement in the liver on hepatobiliary phase gadoxetate disodium-enhanced magnetic resonance images by comparing with the finding of periportal hyperintensity on T2-weighted images and to reveal their clinical significance.Nineteen consecutive patients with diffuse periportal enhancement on hepatobiliary phase images constituted the study population. The intrahepatic diffuse periportal enhancement finding was assessed on whether it corresponded to periportal hyperintense patterns on T2-weighted images or not in the location, and the cases were classified into 2 groups according to this characteristic. Read More

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http://dx.doi.org/10.1097/MD.0000000000014784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6426476PMC
March 2019
1 Read

Simple Magnetic Resonance Scores Associate With Outcomes of Patients With Primary Sclerosing Cholangitis.

Clin Gastroenterol Hepatol 2019 Mar 14. Epub 2019 Mar 14.

Assistance Publique - Hôpitaux de Paris, Sorbonne University, Department of Radiology, Saint-Antoine Hospital, Paris, France.

Background & Aims: Primary sclerosing cholangitis (PSC) has a variable, often progressive course. Magnetic resonance cholangiography (MRC) is used in diagnosis of PSC. Magnetic resonance risk scoring systems, called Anali without and with gadolinium, are used to predict disease progression, determined by radiologic factors. Read More

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http://dx.doi.org/10.1016/j.cgh.2019.03.013DOI Listing
March 2019
2 Reads

Use of Fenofibrate for patients with primary Sclerosing Cholangitis.

Clin Res Hepatol Gastroenterol 2019 Mar 14. Epub 2019 Mar 14.

Division of Hepatology, Department of Medicine, University of Miami, Jackson Medical Towers, 1500 NW, 12th avenue, Suite 1101, Miami, FL, USA. Electronic address:

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http://dx.doi.org/10.1016/j.clinre.2018.11.017DOI Listing
March 2019
1 Read

Liver Transplant After Normothermic Regional Perfusion From Controlled Donors After Circulatory Death: The Norwegian Experience.

Transplant Proc 2019 Mar 28;51(2):475-478. Epub 2019 Jan 28.

Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital, Oslo, Norway; Faculty of Medicine, University of Oslo, Oslo, Norway.

Background: In order to meet the increasing demand for donor organs, the concept of donation after circulatory death (DCD) was reintroduced in Norway, first as a pilot study, followed by the use of DCD as institutional practice. We report the current Norwegian experience with liver transplant after DCD.

Methods: After acceptance from next of kin, life support was withdrawn from patients with devastating brain injury and cardiac arrest observed. Read More

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http://dx.doi.org/10.1016/j.transproceed.2019.01.066DOI Listing
March 2019
2 Reads

Magnetic Resonance Elastography in Primary Sclerosing Cholangitis: Interobserver Agreement for Liver Stiffness Measurement with Manual and Automated Methods.

Acad Radiol 2019 Mar 13. Epub 2019 Mar 13.

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

Rationale And Objective: Primary sclerosing cholangitis, a chronic liver disease causes heterogeneous parenchymal changes and fibrosis. Liver stiffness measurement (LSM) with magnetic resonance Elastography (MRE) may be affected by this heterogeneous distribution. We evaluated interobserver agreement of LSM in primary sclerosing cholangitis (PSC) with manual and automated methods to study the influence of heterogeneous changes. Read More

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http://dx.doi.org/10.1016/j.acra.2019.02.004DOI Listing
March 2019
1 Read

Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: Predictors of Gamma Glutamyltransferase Normalization and Favorable Clinical Course.

J Pediatr 2019 Mar 13. Epub 2019 Mar 13.

Department of Pediatrics, University of Utah, Salt Lake City, UT.

Objective: To investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis.

Study Design: We retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.01.039DOI Listing
March 2019
1 Read
3.790 Impact Factor

[Immune-mediated cholangiopathies : Diagnostic and therapeutic challenges].

Authors:
T Herta U Beuers

Radiologe 2019 Apr;59(4):348-356

Department of Gastroenterology and Hepatology and Tytgat Institute for Liver and Intestinal Research, Amsterdam University Medical Centers, Location Academic Medical Center, AMC, C2-327, Meibergdreef 9, 1105 AZ, Amsterdam, Niederlande.

Background: Immune-mediated cholangiopathies comprise primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and IgG4-associated cholangitis (IAC). A common feature is the progressive destruction of bile ducts leading to cholestasis with fibrosis and cirrhosis of the liver over time. The diseases are mostly identified during routine laboratory testing. Read More

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http://dx.doi.org/10.1007/s00117-019-0513-xDOI Listing
April 2019
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Intestinal dysbiosis augments liver disease progression via NLRP3 in a murine model of primary sclerosing cholangitis.

Gut 2019 Mar 14. Epub 2019 Mar 14.

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

Objective: There is a striking association between human cholestatic liver disease (CLD) and inflammatory bowel disease. However, the functional implications for intestinal microbiota and inflammasome-mediated innate immune response in CLD remain elusive. Here we investigated the functional role of gut-liver crosstalk for CLD in the murine Mdr2 knockout model resembling human primary sclerosing cholangitis (PSC). Read More

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http://dx.doi.org/10.1136/gutjnl-2018-316670DOI Listing
March 2019
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Endoscopic retrograde cholangiopancreatography (ERCP): lessons learned from population-based national registries: a systematic review.

Surg Endosc 2019 Mar 12. Epub 2019 Mar 12.

Department of Surgical and Perioperative Sciences, Umeå University, Umeå, Sweden.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) was introduced more than four decades ago as a diagnostic tool for biliary and pancreatic diseases. Currently, ERCP is mainly used as a therapeutic approach to relieve biliary or pancreatic duct obstruction. Clinical practice has been based on a few large reports and some randomized controlled trials. Read More

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http://dx.doi.org/10.1007/s00464-019-06734-wDOI Listing
March 2019
3 Reads

Secondary sclerosing cholangitis with hemobilia induced by pembrolizumab: Case report and review of published work.

Hepatol Res 2019 Mar 12. Epub 2019 Mar 12.

Third Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

A 66-year-old man was admitted to our department due to cholestatic liver injury. He had received five cycles of pembrolizumab for small-cell lung cancer. Imaging showed the possibility of sclerosing cholangitis (SC) with hemobilia. Read More

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http://doi.wiley.com/10.1111/hepr.13329
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http://dx.doi.org/10.1111/hepr.13329DOI Listing
March 2019
9 Reads

Benign biliary strictures: prevalence, impact, and management strategies.

Clin Exp Gastroenterol 2019 18;12:83-92. Epub 2019 Feb 18.

Department of Gastroenterology and Hepatology, Fiona Stanley Hospital, Murdoch, Perth, WA 6150, Australia,

Benign biliary strictures (BBSs) may form from chronic inflammatory pancreaticobiliary pathologies, postoperative bile-duct injury, or at biliary anastomoses following liver transplantation. Treatment aims to relieve symptoms of biliary obstruction, maintain long-term drainage, and preserve liver function. Endoscopic therapy, including stricture dilatation and stenting, is effective in most cases and the first-line treatment of BBS. Read More

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http://dx.doi.org/10.2147/CEG.S165016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6385742PMC
February 2019
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Chronic Cholangiopathy Associated with Primary Immune Deficiencies Can Be Resolved by Effective Hematopoietic Stem Cell Transplantation.

J Pediatr 2019 Mar 5. Epub 2019 Mar 5.

Immunology Department, Great Ormond Street Hospital, London, United Kingdom.

Objectives: To investigate effects and outcome of hematopoietic stem cell transplantation (HSCT) on sclerosing cholangitis, in pediatric patients with different primary immunodeficiencies (PIDs).

Study Design: From databases in 2 tertiary centers for immunodeficiencies and liver disease, we have identified children with PIDs and sclerosing cholangitis, who have paired clinical, radiologic, and histologic information before and after HSCT and studied their clinical progress and outcome.

Results: Seven of 13 children (53. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223476193003
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http://dx.doi.org/10.1016/j.jpeds.2019.01.015DOI Listing
March 2019
6 Reads

Role of Transplant in Biliary Disease.

Surg Clin North Am 2019 Apr;99(2):387-401

General Surgery, University of Alabama at Birmingham, 1808 7th Avenue South D202 Boshell Building Birmingham, Alabama 35233, USA; Division of Transplantation, University of Alabama at Birmingham, 701 19th Street South 722 Lyons Harrison Birmingham, Alabama 35233. Electronic address:

Orthotopic liver transplantation (OLT) has many roles in biliary disease. OLT provides excellent results for patients with unresectable hilar cholangiocarcinoma. OLT prolongs survival in primary biliary cirrhosis not responsive to therapy and improves quality of life. Read More

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http://dx.doi.org/10.1016/j.suc.2018.12.009DOI Listing
April 2019
2 Reads

Autoimmune Diseases of the Biliary Tract: A Review.

Surg Clin North Am 2019 Apr 10;99(2):185-201. Epub 2019 Feb 10.

Division of Surgical Oncology, Department of Surgery, University of Wisconsin School of Medicine and Public Health, University of Wisconsin, 600 Highland Avenue, Madison, WI 53792, USA. Electronic address:

The management of autoimmune hepatobiliary disorders remains a challenging and emerging area of investigation. An awareness of cholestatic liver diseases is critical to appropriate recognition and management of these challenging diseases, because patients often present asymptomatically, and diagnosis is limited by the lack of disease-specific markers and diagnostic studies. Furthermore, there is a paucity of treatment options because the pathophysiology underlying autoimmune biliary diseases remains largely unknown. Read More

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http://dx.doi.org/10.1016/j.suc.2018.11.003DOI Listing
April 2019
2 Reads

Oral Vancomycin Induces and Maintains Remission of Ulcerative Colitis in the Subset of Patients With Associated Primary Sclerosing Cholangitis.

Inflamm Bowel Dis 2019 Mar 6. Epub 2019 Mar 6.

Department of Gastroenterology, Johns Hopkins School of Medicine, Washington, DC.

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http://dx.doi.org/10.1093/ibd/izz027DOI Listing
March 2019
2 Reads

Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence.

Ann Gastroenterol 2019 Mar-Apr;32(2):124-133. Epub 2019 Jan 15.

Gastroenterology (Nhu An Nguyen, Ryan M. Kwok), Walter Reed National Military Medical Center Bethesda, MD, USA.

Comorbid primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) represent a unique disease phenotype with a different risk profile than PSC or IBD alone. While the pathogenetic mechanisms behind both diseases remain unclear, recent studies have targeted several immune-mediated pathways in an attempt to find a potential therapeutic target. Patients with PSC-associated IBD typically exhibit pancolitis with a right-to-left intestinal inflammatory gradient associated with a greater incidence of backwash ileitis and rectal sparing. Read More

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http://dx.doi.org/10.20524/aog.2019.0344DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394256PMC
January 2019
3 Reads

Frequency of Hepatobiliary Manifestations and Concomitant Liver Disease in Inflammatory Bowel Disease Patients.

Biomed Res Int 2019 31;2019:7604939. Epub 2019 Jan 31.

Department of Life Sciences, Universidade do Estado da Bahia, Rua Silveira Martins 2555, Cabula 41150-000, Salvador, Bahia, Brazil.

Background: In inflammatory bowel disease (IBD) patients there are reports of the occurrence of hepatobiliary manifestations, so the aim of this study was to evaluate the hepatobiliary manifestations in patients with Crohn's disease (CD) and ulcerative colitis (UC) from an IBD reference center.

Methods: Cross-sectional study in an IBD reference center, with interviews and review of medical charts, between July 2015 and August 2016. A questionnaire addressing epidemiological and clinical characteristics was used. Read More

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https://www.hindawi.com/journals/bmri/2019/7604939/
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http://dx.doi.org/10.1155/2019/7604939DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374878PMC
January 2019
9 Reads

Nrf2 ameliorates DDC-induced sclerosing cholangitis and biliary fibrosis and improves the regenerative capacity of the liver.

Toxicol Sci 2019 Mar 2. Epub 2019 Mar 2.

Department of Anatomy and Cell Biology, Medical Faculty, Uniklinik RWTH Aachen University, Aachen, Germany.

The Nrf2 pathway protects against oxidative stress and induces regeneration of various tissues. Here, we investigated whether Nrf2 protects from sclerosing cholangitis and biliary fibrosis and simultaneously induces liver regeneration. Diet containing 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) was fed to Nrf2-KO mice (Nrf2-/-), mice with liver-specific hyper-activated Nrf2 (HKeap1-/-) and wild type (WT) littermates to induce cholangitis, liver fibrosis, and oval cell expansion. Read More

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http://dx.doi.org/10.1093/toxsci/kfz055DOI Listing
March 2019
2 Reads

Molecular Pathogenesis of Cholangiocarcinoma.

BMC Cancer 2019 Feb 28;19(1):185. Epub 2019 Feb 28.

UCL Institute for Liver and Digestive Health, University College London (Royal Free Hospital Campus), Royal Free Hospital, Pond Street, London, NW3 2QG, UK.

Background: Cholangiocarcinomas are a heterogeneous group of malignancies arising from a number of cells of origin along the biliary tree. Although most cases in Western countries are sporadic, large population-based studies have identified a number of risk factors. This review summarises the evidence behind reported risk factors and current understanding of the molecular pathogenesis of cholangiocarcinoma, with a focus on inflammation and cholestasis as the driving forces in cholangiocarcinoma development. Read More

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http://dx.doi.org/10.1186/s12885-019-5391-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394015PMC
February 2019
5 Reads

Inflammation and Neoplasia of the Pouch in Inflammatory Bowel Disease.

Authors:
Freeha Khan Bo Shen

Curr Gastroenterol Rep 2019 Feb 28;21(4):10. Epub 2019 Feb 28.

Department of Gastroenterology/Hepatology/Nutrition, Cleveland Clinic, Cleveland, OH, USA.

Purpose Of Review: Ileal pouch-anal anastomosis (IPAA) is the standard restorative procedure after proctocolectomy in patients with inflammatory bowel disease who require colectomy. The ileal pouch is susceptible to a variety of adverse outcomes including mechanical insult, ischemia, and infectious agents. There is also a risk for developing low-grade dysplasia (LGD), high-grade dysplasia (HGD), or even adenocarcinoma in the pouch. Read More

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http://dx.doi.org/10.1007/s11894-019-0679-4DOI Listing
February 2019
3 Reads

Prevalence of autoimmune diseases and clinical significance of autoantibody profile: Data from National Institute of Hygiene in Rabat, Morocco.

Hum Immunol 2019 Feb 23. Epub 2019 Feb 23.

Laboratory of Human Pathologies Biology, Department of Biology, Faculty of Sciences, and Genomic Center of Human Pathologies, Faculty of Medicine and Pharmacy, Mohammed V University in Rabat, Morocco.

Aim: The objective of this study was to explore the prevalence of various autoimmune diseases (AIDs) in a large cohort of patients and to characterize the autoantibody profile in the patients with and without AIDs to confirm the diagnosis and to refine the Moroccan databases.

Patients And Method: Retrospective study was conducted in the Laboratory of autoimmunity National Institute of Hygiene (NIH) of Rabat in Morocco. A total of 3182 consecutive Moroccan patients (2183 females and 999 males) whose sera were tested for 14 autoantibody profile between 2010 and 2016. Read More

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http://dx.doi.org/10.1016/j.humimm.2019.02.012DOI Listing
February 2019
5 Reads

Liver stiffness measurement by acoustic radiation forced impulse and transient elastography in patients with intrahepatic cholestasis.

Eur J Gastroenterol Hepatol 2019 04;31(4):520-527

Department of Hepato-gastroenetrology and Endemic Medicine, Faculty of Medicine, Cairo University, Cairo, Egypt.

Background: Transient elastography (TE) and acoustic radiation force impulse (ARFI) imaging enable a noninvasive assessment of liver stiffness measurement (LSM) and liver fibrosis/cirrhosis staging. However, their use in cholestatic diseases is still scarce.

Aim: The aim of this study was to evaluate the performance of TE and ARFI for the initial assessment of hepatic fibrosis in intrahepatic cholestatic (IHC) diseases and assess LSM changes after 3 months of specific therapy. Read More

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http://dx.doi.org/10.1097/MEG.0000000000001327DOI Listing

Extraintestinal manifestations in inflammatory bowel disease - epidemiology, genetics, and pathogenesis.

Expert Rev Gastroenterol Hepatol 2019 Apr 20;13(4):307-317. Epub 2019 Feb 20.

a Department of Gastroenterology and Hepatology , University Hospital Zurich , Zurich , Switzerland.

Introduction: Inflammatory bowel disease (IBD) is a chronic inflammatory disorder, primarily of, but not restricted to the gut. Extraintestinal manifestations (EIMs) are frequently observed and involve the joints, eyes, hepatobiliary tract, and skin. Areas covered: In this review, we discuss classical EIM focusing on epidemiology, genetics, and pathogenesis, highlighting recent advances in the understanding of EIM. Read More

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http://dx.doi.org/10.1080/17474124.2019.1574569DOI Listing
April 2019
1 Read

Role of endoscopy in the management of primary sclerosing cholangitis.

World J Gastrointest Endosc 2019 Feb;11(2):84-94

Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States.

Primary sclerosing cholangitis (PSC) is a rare but prominent fibroinflammatory cholangiopathy which can affect individuals of essentially any age. It carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent risk for biliary tract and other complications, and the paucity of effective pharmacotherapies, endoscopy plays a major role in the care of many patients with this disorder. Read More

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http://dx.doi.org/10.4253/wjge.v11.i2.84DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6379747PMC
February 2019
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CCL21-expression and accumulation of CCR7 NK cells in livers of patients with primary sclerosing cholangitis.

Eur J Immunol 2019 Feb 20. Epub 2019 Feb 20.

Research Department of Virus Immunology, Heinrich Pette Institute, Hamburg, Germany.

The pathogenesis of primary sclerosing cholangitis (PSC), an autoimmune liver disease, remains unknown. The aim of this study was to characterize peripheral blood and intrahepatic NK cells from patients with PSC. Peripheral blood samples from patients with PSC, other autoimmune liver diseases, and from healthy control individuals were used, as well as liver tissues from PSC patients undergoing liver transplantation. Read More

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http://dx.doi.org/10.1002/eji.201847965DOI Listing
February 2019
3 Reads

Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies.

World J Gastroenterol 2019 Feb;25(6):659-671

Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States.

Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by progressive fibroinflammatory destruction of the intra- and/or extrahepatic biliary ducts. While its features and disease course can be variable, most patients with PSC have concurrent inflammatory bowel disease and will eventually develop liver cirrhosis and end-stage liver disease, with liver transplantation representing the only potentially curative option. Importantly, PSC is associated with a significantly increased risk of malignancy compared to the general population, mainly cholangiocarcinoma, gallbladder carcinoma, hepatocellular carcinoma, and colorectal cancer, with nearly 50% of deaths in patients with PSC being due to cancer. Read More

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http://dx.doi.org/10.3748/wjg.v25.i6.659DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378537PMC
February 2019
2 Reads

Evolving role of magnetic resonance techniques in primary sclerosing cholangitis.

World J Gastroenterol 2019 Feb;25(6):644-658

Oxford Centre for Clinical Magnetic Resonance Research, Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom.

Development of non-invasive methods to risk-stratify patients and predict clinical endpoints have been identified as one of the key research priorities in primary sclerosing cholangitis (PSC). In addition to serum and histological biomarkers, there has been much recent interest in developing imaging biomarkers that can predict disease course and clinical outcomes in PSC. Magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) continue to play a central role in the diagnosis and follow-up of PSC patients. Read More

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http://dx.doi.org/10.3748/wjg.v25.i6.644DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378540PMC
February 2019
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[Clinicopathological characteristics and diagnosis of IgG4related sialadenitis].

Beijing Da Xue Xue Bao Yi Xue Ban 2019 Feb;51(1):1-3

Department of Oral Radiology, Peking University School and Hospital of Stomatology, Beijing 100081, China.

Immunoglobulin G4-related sialadenitis (IgG4-RS) is a newly recognized immune-mediated disease and one of immunoglobulin G4-related diseases (IgG4-RD). Our multidisciplinary research group investigated the clinicopathological characteristics and diagnosis of IgG4-RS during the past 10 years. Clinically, it showed multiple bilateral enlargement of major salivary glands (including sublingual and accessory parotid glands) and lacrimal glands. Read More

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http://dx.doi.org/10.19723/j.issn.1671-167X.2019.01.001DOI Listing
February 2019
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Predictive criteria of response to endoscopic treatment for severe strictures in primary sclerosing cholangitis.

Clin Res Hepatol Gastroenterol 2019 Feb 13. Epub 2019 Feb 13.

Department of Radiology, Saint-Antoine Hospital, Assistance Publique-Hôpitaux de Paris AP-HP, Sorbonne university, 75012 Paris, France. Electronic address:

Background: The aim of this study was to identify predictive criteria of improvement after endoscopic treatment (ET) for severe strictures of extrahepatic bile ducts in patients with primary sclerosing cholangitis (PSC).

Methods: PSC patients who had at least one ET for severe stricture were included. Features of magnetic resonance cholangiography (MRC), performed before ET, were evaluated according to a standard model of interpretation, and a radiologic qualitative score of probability of improvement after ET was built. Read More

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http://dx.doi.org/10.1016/j.clinre.2019.01.003DOI Listing
February 2019
2 Reads