The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
Keith D. Lindor - One of the best experts on this subject based on the ideXlab platform.
-
acg clinical guideline primary Sclerosing Cholangitis
The American Journal of Gastroenterology, 2015Co-Authors: Keith D. Lindor, Kris V. Kowdley, Edwyn M HarrisonAbstract:Primary Sclerosing Cholangitis is a chronic cholestatic liver disease that can shorten life and may require liver transplantation. The cause is unknown, although it is commonly associated with colitis. There is no approved or proven therapy, although ursodeoxycholic acid is used by many on an empiric basis. Complications including portal hypertension, fat-soluble vitamin deficiency, metabolic bone diseases, and development of cancers of the bile duct or colon can occur.
-
primary Sclerosing Cholangitis
The Lancet, 2013Co-Authors: Gideon M. Hirschfield, Tom H. Karlsen, Keith D. Lindor, David H AdamsAbstract:Summary Primary Sclerosing Cholangitis is the classic hepatobiliary manifestation of inflammatory bowel disease and is generally chronic and progressive. Patients frequently present with asymptomatic, anicteric cholestasis, but many develop progressive biliary strictures with time, leading to recurrent Cholangitis, biliary cirrhosis, and end-stage liver disease. Medical treatment does not slow the progression of disease, and many patients need liver transplantation, after which recurrent disease is a risk. The increased incidence of hepatobiliary cancer, which is not related to the underlying severity of biliary fibrosis, is of particular concern. Risk of colorectal cancer is also increased in patients with coexistent inflammatory bowel disease. Mechanistic insights have arisen from studies of secondary Sclerosing Cholangitis, in which a similar clinical profile is associated with a specific cause, and genomic studies have elucidated potential disease-initiating pathways in the primary form. The close association between inflammatory bowel disease and primary Sclerosing Cholangitis underscores the need to further understand the role of environmental factors in generation of lymphocytes that are postulated to be retargeted, deleteriously, to the biliary tree. Treatment of primary Sclerosing Cholangitis is confined to supportive measures, but advances in pathobiology suggest that new stratified approaches will soon be available.
-
primary Sclerosing Cholangitis patients with serial polysomy fluorescence in situ hybridization results are at increased risk of cholangiocarcinoma
The American Journal of Gastroenterology, 2011Co-Authors: Benjamin R. Kipp, Keith D. Lindor, Jesse S. Voss, Amy C. Clayton, Kevin C. Halling, Gregory J. GoresAbstract:Primary Sclerosing Cholangitis Patients With Serial Polysomy Fluorescence In Situ Hybridization Results Are at Increased Risk of Cholangiocarcinoma
-
alkaline phosphatase normalization is associated with better prognosis in primary Sclerosing Cholangitis
Digestive and Liver Disease, 2011Co-Authors: Peter P Stanich, Andrea A. Gossard, Einar Bjornsson, Felicity Enders, Roberta A Jorgensen, Keith D. LindorAbstract:Background Primary Sclerosing Cholangitis results in elevated but fluctuating serum alkaline phosphatase levels that occasionally return to normal.
-
minocycline in the treatment of patients with primary Sclerosing Cholangitis results of a pilot study
The American Journal of Gastroenterology, 2009Co-Authors: Marina G Silveira, Andrea A. Gossard, Roberta A Jorgensen, Natalie J Torok, Jill C Keach, R Janice N L Petz, Keith D. LindorAbstract:Minocycline in the Treatment of Patients With Primary Sclerosing Cholangitis: Results of a Pilot Study
Tom H. Karlsen - One of the best experts on this subject based on the ideXlab platform.
-
primary Sclerosing Cholangitis a comprehensive review
Journal of Hepatology, 2017Co-Authors: Tom H. Karlsen, Trine Folseraas, Douglas Thorburn, Mette VesterhusAbstract:Primary Sclerosing Cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and a lack of proven surveillance strategies, patients currently have significant unmet needs. In the present seminar, we provide a comprehensive review of the status of the field. We emphasise developments related to patient stratification and disease behaviour, and provide an overview of management options from a practical, patient-centered perspective. We survey advances made in the understanding of PSC pathogenesis and summarise the ongoing efforts to develop an effective therapy based on these insights.
-
characterization of animal models for primary Sclerosing Cholangitis psc
Journal of Hepatology, 2014Co-Authors: Peter Fickert, Gideon M. Hirschfield, Christoph Schramm, Hannsulrich Marschall, Marion J Pollheimer, Ulrich Beuers, Carolin Lackner, Chantal Housset, Verena Keitel, Tom H. KarlsenAbstract:Primary Sclerosing Cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC.
-
primary Sclerosing Cholangitis
The Lancet, 2013Co-Authors: Gideon M. Hirschfield, Tom H. Karlsen, Keith D. Lindor, David H AdamsAbstract:Summary Primary Sclerosing Cholangitis is the classic hepatobiliary manifestation of inflammatory bowel disease and is generally chronic and progressive. Patients frequently present with asymptomatic, anicteric cholestasis, but many develop progressive biliary strictures with time, leading to recurrent Cholangitis, biliary cirrhosis, and end-stage liver disease. Medical treatment does not slow the progression of disease, and many patients need liver transplantation, after which recurrent disease is a risk. The increased incidence of hepatobiliary cancer, which is not related to the underlying severity of biliary fibrosis, is of particular concern. Risk of colorectal cancer is also increased in patients with coexistent inflammatory bowel disease. Mechanistic insights have arisen from studies of secondary Sclerosing Cholangitis, in which a similar clinical profile is associated with a specific cause, and genomic studies have elucidated potential disease-initiating pathways in the primary form. The close association between inflammatory bowel disease and primary Sclerosing Cholangitis underscores the need to further understand the role of environmental factors in generation of lymphocytes that are postulated to be retargeted, deleteriously, to the biliary tree. Treatment of primary Sclerosing Cholangitis is confined to supportive measures, but advances in pathobiology suggest that new stratified approaches will soon be available.
-
Update on primary Sclerosing Cholangitis
Journal of hepatology, 2013Co-Authors: Tom H. Karlsen, Kirsten Muri BobergAbstract:Primary Sclerosing Cholangitis (PSC) remains one of the most challenging conditions of clinical hepatology. There has been a steady growth in research to overcome this fact and the present review aims at summarizing the most recently published literature. The main emphasis will be put on the link of recent pathogenetic insights to clinical characteristics and patient management. With regard to pathogenesis, there is no consensus yet as to whether immune mediated injury or factors related to bile acid physiology are the most important. It also remains to be clarified whether PSC is a mixed bag of various secondary etiologies yet to be defined, or a disease entity predominantly represented by Sclerosing Cholangitis in the context of inflammatory bowel disease. Most important, there is no available medical therapy with proven influence on clinical end points, and timing of liver transplantation and patient follow-up are challenging due to the unpredictable and high risk of cholangiocarcinoma.
Mark G Swain - One of the best experts on this subject based on the ideXlab platform.
-
reversible Sclerosing Cholangitis secondary to cryptosporidiosis in a renal transplant patient
Journal of Hepatology, 2003Co-Authors: Ayman A Abdo, John Klassen, Stefan J Urbanski, Earl L Raber, Mark G SwainAbstract:Cryptosporidium parvum is a well-known cause of chronic diarrhea. In human immunodeficiency virus (HIV)-infected patients as well as in other immunocompromised patients it has also been shown to cause Sclerosing Cholangitis. We report a case of reversible C. parvum-induced Sclerosing Cholangitis in a renal transplant patient. This 40-year-old female received a renal transplant 9 years prior to presentation. She had no history of liver disease and was doing well on tacrolimus, prednisone, and azathioprine. She developed diarrhea and was found to have C. parvum present in the stool. Shortly after, she developed clinical, biochemical, radiologic, and histologic features of SC. After accidental reduction in her immunesuppression secondary to starting her on rifampin to treat her itching, she cleared C. parvum from her stool and had a marked improvement in her diarrhea, jaundice, and general health. Her liver enzymes normalized and magnetic resonance cholangiography showed complete resolution of biliary abnormalities. To our knowledge, this is the first case of C. parvum-induced Sclerosing Cholangitis in a renal transplant patient and one of a few in non-HIV patients. It is also the first to document resolution of Sclerosing Cholangitis after eradication of C. parvum in a non-HIV patient.
-
case report reversible Sclerosing Cholangitis secondary to cryptosporidiosis in a renal transplant patient
2003Co-Authors: Ayman A Abdo, John Klassen, Stefan J Urbanski, Earl L Raber, Mark G SwainAbstract:Cryptosporidium parvum is a well-known cause of chronic diarrhea. In human immunodeficiency virus (HIV)infected patients as well as in other immunocompromised patients it has also been shown to cause Sclerosing Cholangitis. We report a case of reversible C. parvum-induced Sclerosing Cholangitis in a renal transplant patient. This 40-year-old female received a renal transplant 9 years prior to presentation. She had no history of liver disease and was doing well on tacrolimus, prednisone, and azathioprine. She developed diarrhea and was found to have C. parvum present in the stool. Shortly after, she developed clinical, biochemical, radiologic, and histologic features of SC. After accidental reduction in her immunesuppression secondary to starting her on rifampin to treat her itching, she cleared C. parvum from her stool and had a marked improvement in her diarrhea, jaundice, and general health. Her liver enzymes normalized and magnetic resonance cholangiography showed complete resolution of biliary abnormalities. To our knowledge, this is the first case of C. parvum-induced Sclerosing Cholangitis in a renal transplant patient and one of a few in non-HIV patients. It is also the first to document resolution of Sclerosing Cholangitis after eradication of C. parvum in a non-HIV patient. q 2003 European Association for the Study of the Liver. Published by Elsevier Science B.V. All rights reserved.
Gregory J. Gores - One of the best experts on this subject based on the ideXlab platform.
-
primary Sclerosing Cholangitis what the gastroenterologist and hepatologist needs to know
Clinics in Liver Disease, 2017Co-Authors: Andrea A. Gossard, Gregory J. GoresAbstract:Primary Sclerosing Cholangitis (PSC) is a chronic, idiopathic biliary tract disease characterized by segmental strictures. The disease is progressive with no proven treatments and may eventually lead to cirrhosis and end-stage liver disease. Abrupt changes in liver biochemistries, pain, and/or Cholangitis may suggest a dominant stricture amenable to endoscopic therapy or the development of cholangiocarcinoma. Patients with PSC are at increased risk of cholangiocarcinoma. There is a strong association with inflammatory bowel disease, and an associated increased risk of colorectal cancer. Colonoscopy every 1 to 2 years is appropriate.
-
biliary multifocal chromosomal polysomy and cholangiocarcinoma in primary Sclerosing Cholangitis
The American Journal of Gastroenterology, 2015Co-Authors: John E. Eaton, James H. Tabibian, Emily G. Barr Fritcher, Benjamin R. Kipp, Kevin C. Halling, Gregory J. Gores, Elizabeth J. Atkinson, Mark Topazian, Andrea A. Gossard, Konstantinos N. LazaridisAbstract:Biliary Multifocal Chromosomal Polysomy and Cholangiocarcinoma in Primary Sclerosing Cholangitis
-
primary Sclerosing Cholangitis patients with serial polysomy fluorescence in situ hybridization results are at increased risk of cholangiocarcinoma
The American Journal of Gastroenterology, 2011Co-Authors: Benjamin R. Kipp, Keith D. Lindor, Jesse S. Voss, Amy C. Clayton, Kevin C. Halling, Gregory J. GoresAbstract:Primary Sclerosing Cholangitis Patients With Serial Polysomy Fluorescence In Situ Hybridization Results Are at Increased Risk of Cholangiocarcinoma
-
the value of serum ca 19 9 in predicting cholangiocarcinomas in patients with primary Sclerosing Cholangitis
Digestive Diseases and Sciences, 2005Co-Authors: Cynthia Levy, Nicholas F. Larusso, Keith D. Lindor, Gregory J. Gores, James F Lymp, Paul AnguloAbstract:CA 19-9 has been used with questionable accuracy to aid diagnosis of cholangiocarcinoma complicating primary Sclerosing Cholangitis. We aimed to characterize the test properties of CA 19-9 and of a change in CA 19-9 over time in predicting cholangiocarcinoma. Charts of 208 patients were reviewed. Fourteen patients had cholangiocarcinoma. Median CA 19-9 was higher with cholangiocarcinoma (15 vs. 290 U/ml, p < 0.0001). A cutoff of 129 U/ml provided: sensitivity 78.6%, specificity 98.5%, adjusted positive predictive value 56.6% and negative predictive value 99.4%. The median change over time was 664 U/ml in cholangiocarcinoma compared to 6.7 U/ml in primary Sclerosing Cholangitis alone (p < 0.0001). A cutoff of 63.2 U/ml for change in CA 19-9 provided: sensitivity 90%, specificity 98% and positive predictive value 42%. Only 2 patients with cholangiocarcinoma were the candidates for curative therapy. In conclusion, the positive predictive value of an elevated CA 19-9 was 56.6%; only advanced cases were detected by this method.
-
the utility of ca 19 9 in the diagnoses of cholangiocarcinoma in patients without primary Sclerosing Cholangitis
The American Journal of Gastroenterology, 2000Co-Authors: Anand Patel, Nicholas F. Larusso, Denise M Harnois, George G Klee, Gregory J. GoresAbstract:The utility of CA 19-9 in the diagnoses of cholangiocarcinoma in patients without primary Sclerosing Cholangitis
Stefan J Urbanski - One of the best experts on this subject based on the ideXlab platform.
-
the burden of large and small duct primary Sclerosing Cholangitis in adults and children a population based analysis
The American Journal of Gastroenterology, 2007Co-Authors: Gilaad G. Kaplan, Stefan J Urbanski, Kevin B Laupland, Decker Butzner, Samuel S LeeAbstract:The Burden of Large and Small Duct Primary Sclerosing Cholangitis in Adults and Children: A Population-Based Analysis
-
reversible Sclerosing Cholangitis secondary to cryptosporidiosis in a renal transplant patient
Journal of Hepatology, 2003Co-Authors: Ayman A Abdo, John Klassen, Stefan J Urbanski, Earl L Raber, Mark G SwainAbstract:Cryptosporidium parvum is a well-known cause of chronic diarrhea. In human immunodeficiency virus (HIV)-infected patients as well as in other immunocompromised patients it has also been shown to cause Sclerosing Cholangitis. We report a case of reversible C. parvum-induced Sclerosing Cholangitis in a renal transplant patient. This 40-year-old female received a renal transplant 9 years prior to presentation. She had no history of liver disease and was doing well on tacrolimus, prednisone, and azathioprine. She developed diarrhea and was found to have C. parvum present in the stool. Shortly after, she developed clinical, biochemical, radiologic, and histologic features of SC. After accidental reduction in her immunesuppression secondary to starting her on rifampin to treat her itching, she cleared C. parvum from her stool and had a marked improvement in her diarrhea, jaundice, and general health. Her liver enzymes normalized and magnetic resonance cholangiography showed complete resolution of biliary abnormalities. To our knowledge, this is the first case of C. parvum-induced Sclerosing Cholangitis in a renal transplant patient and one of a few in non-HIV patients. It is also the first to document resolution of Sclerosing Cholangitis after eradication of C. parvum in a non-HIV patient.
-
case report reversible Sclerosing Cholangitis secondary to cryptosporidiosis in a renal transplant patient
2003Co-Authors: Ayman A Abdo, John Klassen, Stefan J Urbanski, Earl L Raber, Mark G SwainAbstract:Cryptosporidium parvum is a well-known cause of chronic diarrhea. In human immunodeficiency virus (HIV)infected patients as well as in other immunocompromised patients it has also been shown to cause Sclerosing Cholangitis. We report a case of reversible C. parvum-induced Sclerosing Cholangitis in a renal transplant patient. This 40-year-old female received a renal transplant 9 years prior to presentation. She had no history of liver disease and was doing well on tacrolimus, prednisone, and azathioprine. She developed diarrhea and was found to have C. parvum present in the stool. Shortly after, she developed clinical, biochemical, radiologic, and histologic features of SC. After accidental reduction in her immunesuppression secondary to starting her on rifampin to treat her itching, she cleared C. parvum from her stool and had a marked improvement in her diarrhea, jaundice, and general health. Her liver enzymes normalized and magnetic resonance cholangiography showed complete resolution of biliary abnormalities. To our knowledge, this is the first case of C. parvum-induced Sclerosing Cholangitis in a renal transplant patient and one of a few in non-HIV patients. It is also the first to document resolution of Sclerosing Cholangitis after eradication of C. parvum in a non-HIV patient. q 2003 European Association for the Study of the Liver. Published by Elsevier Science B.V. All rights reserved.
