Scientific Research
This page is in a'blog' format with the latest scientific PSC research identified by Google Scholar, Readbyqmx.com and other sources added regularly.

John M Fell, Rafeeq Muhammed, Chris Spray, Kay Crook, Richard K Russell, BSPGHAN IBD working group

Abstract
​Ulcerative colitis (UC) in children is increasing. The range of treatments available has also increased too but around 1 in 4 children still require surgery to control their disease. An up-to-date understanding of treatments is essential for all clinicians involved in the care of UC patients to ensure appropriate and timely treatment while minimising the risk of complications and side effects.

Please click here to read the full article

Arch Dis Child doi:10.1136/archdischild-2014-307218

Primary sclerosing cholangitis during childhood and adolescence

Tamir Miloh and Pinar Bulut, Clinical Liver Disease Special Issue: Liver Disease in Children
Volume 2, Issue 5, pages 215–218, October 2013

Primary sclerosing cholangitis (PSC) is an idiopathic, chronic hepatobiliary disorder characterized by inflammation with progressive obliterative fibrosis and focal dilatation of the intrahepatic and/or extrahepatic bile ducts which leads to biliary cirrhosis and end-stage liver disease.[1] The clinical features of PSC in children and adolescents are summarized in Table 2. PSC is more common in Caucasian male patients and has a strong association with inflammatory bowel disease (IBD), especially ulcerative colitis. In children, secondary causes of sclerosing cholangitis include Langerhans cell histiocytosis, immunodeficiencies, and cystic fibrosis. The incidence of PSC in children is 0.23 cases per 100,000 person-years compared with 1.11/100,000 in adults.[2] . . . Please click here to access the full text of this article. There is also a video presentation and an interview with the authors

Randomised clinical trial: vancomycin or metronidazole in patients with primary sclerosing cholangitis - a pilot study

J. H. Tabibian, E. Weeding, R. A. Jorgensen, J. L. Petz, J. C. Keach, J. A. Talwalkar, K. D. Lindor
Alimentary Pharmacology & Therapeutics Volume 37, Issue 6, pages 604–612, March 2013

Summary

Background
Emerging data suggest that oral antibiotics may have therapeutic effects in primary sclerosing cholangitis (PSC), but published studies are limited.

Aims
To investigate the safety and efficacy of oral vancomycin and metronidazole in patients with PSC.

Methods
Thirty-five patients with PSC were randomised in a double-blind manner into four groups: vancomycin 125 mg or 250 mg four times/day, or metronidazole 250 mg or 500 mg three times/day for 12 weeks. The primary endpoint was decrease in alkaline phosphatase (ALK) at 12 weeks. Secondary end points included serum bilirubin and Mayo PSC risk score; pruritus; and adverse effects (AEs). Nonparametric tests were used for analysis.

Results
The primary endpoint was reached in the low-dose (−43% change in ALK, P = 0.03) and high-dose (−40%, P = 0.02) vancomycin groups, with two patients in the former experiencing ALK normalisation . . . Please click here to read the full text of this article

Note: The reference list of the article provides links to other full text articles.

Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin


Yinka K. Davies, Cynthia J. Tsay, Dario V. Caccamo, Kathleen M. Cox, Ricardo O. Castillo, and Kenneth L. Cox
Case Reports in Transplantation
Volume 2013 (2013), Article ID 314292, 5 pages
http://dx.doi.org/10.1155/2013/314292

Abstract

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin ... Please click here to access to the full text of this article

Systemic therapy for cholangiocarcinoma

Saeed Sadeghi and Richard S. Finn. Clinical Liver Disease, 2014, Special Issue: Autoimmune Liver Disease, Part 4, Volume 3, Issue 4, pages 86–89

Abstract

Cholangiocarcinoma (CCA) is an increasingly common biliary malignancy and accounting for 3% of all gastrointestinal tumors. In the United States, the incidence is 1 to 2 cases per 100,000 population and is uncommon compared with other cancer types. While the incidence of intrahepatic CCAs has been rising in industrialized countries, the incidence of extrahepatic CCA is declining worldwide.1 CCA continues to have a high mortality, with a 5-year survival rate of 10%.[2] In the Western world, the most commonly associated risk factor for CCA is primary sclerosing cholangitis, with an annual risk of 0.5%-1.5%  . . . Please click here to read the full text of this article

Endoscopic management of primary sclerosing cholangiti

Ryan Law D.O. and Todd H. Baron M.D., Clinical Liver Disease: Special Issue: Autoimmune Liver Disease, Part 4, Volume 3, Issue 4, pages 79–82, April 2014

Abstract

Primary sclerosing cholangitis (PSC) is a progressive, chronic fibroinflammatory disorder involving the intra- and extrahepatic biliary tree that can lead to cirrhosis and liver failure in a subset of patients. Additionally, progression of PSC can lead to life-threatening episodes of ascending cholangitis and the development of cholangiocarcinoma (CCA). Herein we describe the endoscopic evaluation and therapeutic management of PSC and CCA . . . Please click here to read the full article

Update on inflammatory bowel disease in patients with primary sclerosing cholangitis

Christos Tsaitas, Anysia Semertzidou and Emmanouil Sinakos. World J Hepatol. 2014 April 27; 6(4): 1780-187. Published online 2014 April 27. doi: 10.4254/wjh.v6.i4.178.

Abstract

Patients with primary sclerosing cholangitis (PSC) complicated by inflammatory bowel disease (IBD) represent a distinct subset of patients with unique characteristics, which have serious clinical implications. The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective. The prevalence of IBD, mainly ulcerative colitis in PSC patients is estimated to be 21%-80%, dependent on screening programs and nationality . . .  Please click here to read further

Medical management of primary sclerosing cholangitis

Atoosa Rabiee M.D., Cynthia Levy M.D

Primary sclerosing cholangitis (PSC) is characterized by inflammation, fibrosis, and stricturing of intrahepatic and/or extrahepatic bile ducts, eventually progressing to biliary cirrhosis and end-stage liver disease. Two-thirds of affected patients are males, with the median age at presentation of 40 years.[1] Although clinical presentation varies significantly, serum alkaline phosphatase (ALP) is usually elevated at the time of diagnosis, and 60%-80% of patients have associated inflammatory bowel disease (IBD). In addition, there is a strong association with increased risk of hepatobiliary and colonic neoplasias . . .  To read further please click on the title where you will be taken to the original site with the full article available to read.

Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis

Ewa Wunsch, Jocelyn Trottier, Malgorzata Milkiewicz, Joanna Raszeja-Wyszomirska, Gideon M Hirschfield, Olivier Barbier, Piotr Milkiewicz. Hepatology: Official Journal of the American Association for the Study of Liver Diseases, 2014, February 12

Ursodeoxycholic acid (UDCA) is no longer recommended for the management of adult patients with primary sclerosing cholangitis (PSC). We undertook prospective evaluation of UDCA withdrawal in a group of consecutive patients with PSC. Twenty six patients, all treated with UDCA (dose range 10-15mg/kg/day) were included. Paired blood samples for liver biochemistry, bile acids, fibroblast growth factor 19 (FGF19) were collected before UDCA withdrawal and 3 months later . . . To read further, please click on the title.

Researchers explore transplanting stem cells to reduce inflammation in the liver

University of Birmingham researchers are to lead a worldwide collaboration of scientists looking at the possibility of transplanting stem cells from one person to another to reduce inflammation in the liver.

Prof Philip Newsome and Dr Gideon Hirschfield, from the University's School of Immunity and Infection, will lead the €5.4 million Mesenchymal stem cells (MSC) to Reduce Liver Inflammation (MERLIN) programme which will include the first clinical trial of mesenchymal stem cells in liver disease in the UK.

The EU-funded programme will study how mesenchymal stem cells can reduce inflammation in the liver of people suffering from primary sclerosing cholangitis (PSC), a disease which causes inflammation and thickening of the bile ducts, build-up of bile in the liver and life-threatening liver disease . . . To read further, please click on the title.

Provided by University of BirminghamReference: http://phys.org/wire-news/156668415/researchers-explore-transplanting-stem-cells-to-reduce-inflammat.html (accessed 22.03.14)