sclerosing cholangitis

Summary

Summary: Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.

Top Publications

  1. pmc Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal
    Kazuichi Okazaki
    The Third Department of Internal Medicine, Kansai Medical University, Moriguchi, Japan
    J Gastroenterol 41:626-31. 2006
  2. ncbi Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic
    Yinka K Davies
    Department of Pediatrics, Lucile Packard Children s Hospital, Stanford University, Palo Alto, CA 94304, USA
    J Pediatr Gastroenterol Nutr 47:61-7. 2008
  3. ncbi Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients
    Cornelia M Gelbmann
    Department of Internal Medicine I and Institute of Pathology, University of Regensburg, Regensburg, Germany
    Am J Gastroenterol 102:1221-9. 2007
  4. ncbi Primary sclerosing cholangitis in children: a long-term follow-up study
    Ariel E Feldstein
    Division of Gastroenterology and Hepatology, Department of Pediatric and Adolescent Medicine, Mayo Medical School, Clinic and Foundation, Rochester, MN 55905, USA
    Hepatology 38:210-7. 2003
  5. ncbi Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study
    G V Gregorio
    Department of Child Health, Institute of Liver Studies, and Department of Radiology, King's College Hospital, London, England
    Hepatology 33:544-53. 2001
  6. ncbi Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis
    Flavia D Mendes
    Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Am J Gastroenterol 101:2070-5. 2006
  7. ncbi IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?
    Yoh Zen
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Am J Surg Pathol 28:1193-203. 2004
  8. ncbi Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis
    Darrell S Pardi
    Division of Gastroenterology and Hepatology and Division of Biostatistics, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Gastroenterology 124:889-93. 2003
  9. ncbi High lifetime risk of cancer in primary sclerosing cholangitis
    Marian M H Claessen
    Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands
    J Hepatol 50:158-64. 2009
  10. ncbi Natural history and prognostic variables in primary sclerosing cholangitis
    J M Farrant
    Department of Radiology, King s College Hospital, Denmark Hill, London, England
    Gastroenterology 100:1710-7. 1991

Detail Information

Publications263 found, 100 shown here

  1. pmc Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal
    Kazuichi Okazaki
    The Third Department of Internal Medicine, Kansai Medical University, Moriguchi, Japan
    J Gastroenterol 41:626-31. 2006
  2. ncbi Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic
    Yinka K Davies
    Department of Pediatrics, Lucile Packard Children s Hospital, Stanford University, Palo Alto, CA 94304, USA
    J Pediatr Gastroenterol Nutr 47:61-7. 2008
    Primary sclerosing cholangitis is a rare chronic cholestatic condition of unknown etiology, frequently associated with inflammatory bowel disease and characterized by diffuse fibrosing and inflammatory destruction of the intra- and/or ..
  3. ncbi Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients
    Cornelia M Gelbmann
    Department of Internal Medicine I and Institute of Pathology, University of Regensburg, Regensburg, Germany
    Am J Gastroenterol 102:1221-9. 2007
    b>Sclerosing cholangitis in critically ill patients (SC-CIP) is a newly described entity of severe biliary disease with progression to liver cirrhosis...
  4. ncbi Primary sclerosing cholangitis in children: a long-term follow-up study
    Ariel E Feldstein
    Division of Gastroenterology and Hepatology, Department of Pediatric and Adolescent Medicine, Mayo Medical School, Clinic and Foundation, Rochester, MN 55905, USA
    Hepatology 38:210-7. 2003
    Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain...
  5. ncbi Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study
    G V Gregorio
    Department of Child Health, Institute of Liver Studies, and Department of Radiology, King's College Hospital, London, England
    Hepatology 33:544-53. 2001
    To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver ..
  6. ncbi Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis
    Flavia D Mendes
    Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Am J Gastroenterol 101:2070-5. 2006
    Biliary strictures, similar to primary sclerosing cholangitis (PSC), have been reported in patients with autoimmune pancreatitis, which is characterized by elevated serum IgG4 levels and responsiveness to corticosteroids...
  7. ncbi IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?
    Yoh Zen
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
    Am J Surg Pathol 28:1193-203. 2004
    b>Sclerosing cholangitis (SC) is a heterogeneous disease entity. Different etiologies such as choledocholithiasis, biliary tumor, or pericholangitis can manifest as SC...
  8. ncbi Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis
    Darrell S Pardi
    Division of Gastroenterology and Hepatology and Division of Biostatistics, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Gastroenterology 124:889-93. 2003
    ..decrease the risk for developing colorectal dysplasia in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC)...
  9. ncbi High lifetime risk of cancer in primary sclerosing cholangitis
    Marian M H Claessen
    Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands
    J Hepatol 50:158-64. 2009
    Primary sclerosing cholangitis (PSC) patients are at risk for developing cholangiocarcinoma (CCA) and colorectal carcinoma (CRC). Our aim was to assess the risk of malignancies and their influence on survival.
  10. ncbi Natural history and prognostic variables in primary sclerosing cholangitis
    J M Farrant
    Department of Radiology, King s College Hospital, Denmark Hill, London, England
    Gastroenterology 100:1710-7. 1991
    The clinical features at the time of presentation and the outcome in 126 patients with primary sclerosing cholangitis were studied to clarify the natural history and prognosis in symptomatic and asymptomatic individuals...
  11. ncbi Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis
    Itaru Naitoh
    Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Mizuho ku, Nagoya, Japan
    J Gastroenterol 44:1147-55. 2009
    IgG4-related sclerosing cholangitis (IgG4-SC) is one of the diseases associated with autoimmune pancreatitis. Several cases of IgG4-SC showed no pancreas abnormalities and it was difficult to distinguish cholangiocarcinoma...
  12. ncbi Sclerosing cholangitis following severe trauma: description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms
    Johannes Benninger
    Department of Medicine I, Friedrich Alexander University Erlangen Nuremberg, Ulmenweg 18, Erlangen D 91054, Germany
    World J Gastroenterol 11:4199-205. 2005
    ..Persistent cholestasis is a rare complication of severe trauma or infections. Little is known about the possible pathomechanisms and the clinical course...
  13. ncbi Sclerosing cholangitis and liver cirrhosis after extrabiliary infections: report on three cases
    W Scheppach
    Department of Medicine, The University of Wuerzburg, Wuerzburg, Germany
    Crit Care Med 29:438-41. 2001
    To describe three unusual cases of sclerosing cholangitis after severe extrahepatic/extrabiliary bacterial infections.
  14. ncbi IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis
    Lizhi Zhang
    Department of Laboratory Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Am J Surg Pathol 34:88-94. 2010
    b>Sclerosing cholangitis can be primary (PSC) or secondary. One unusual cause of secondary sclerosing cholangitis is the newly recognized entity of IgG4-associated cholangitis...
  15. ncbi Secondary sclerosing cholangitis
    Petra Ruemmele
    Department of Internal Medicine I, University of Regensburg, Germany
    Nat Rev Gastroenterol Hepatol 6:287-95. 2009
    Secondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and progressive destruction of the biliary tree that leads to ..
  16. pmc High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis
    Keith D Lindor
    Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USA
    Hepatology 50:808-14. 2009
    ..trials are inconclusive regarding the efficacy of ursodeoxycholic acid (UDCA) for treating primary sclerosing cholangitis (PSC)...
  17. ncbi Signal transducer and activator of transcription 3 protects from liver injury and fibrosis in a mouse model of sclerosing cholangitis
    Markus Mair
    Ludwig Boltzmann Institute for Cancer Research, Vienna, Austria
    Gastroenterology 138:2499-508. 2010
    ..3 (Stat3) is the main mediator of interleukin-6-type cytokine signaling required for hepatocyte proliferation and hepatoprotection, but its role in sclerosing cholangitis and other cholestatic liver diseases remains unresolved.
  18. ncbi Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis
    Takahiro Nakazawa
    Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho cho, Mizuho ku, Nagoya 467 8601, Japan
    Gastrointest Endosc 60:937-44. 2004
    b>Sclerosing cholangitis with autoimmune pancreatitis has a cholangiographic appearance that is similar to that of primary sclerosing cholangitis, but only the former responds well to corticosteroid therapy...
  19. ncbi Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis
    Yoh Zen
    Department of Human Pathology, Kanazawa University Graduate School of Medicine, 13 1 Takarama chi, Kanazawa 920 8640, Japan
    Hepatology 45:1538-46. 2007
    ..7-fold), IL-10/CD4 (45.3-fold), and tumor growth factor (TGF)-beta/CD4 (39.4-fold) than did primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC)...
  20. ncbi Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community
    Kiran Bambha
    Division of Gastroenterology and Hepatology, Mayo Medical School, Clinic and Foundation, 200 First Street SW, Rochester, MN 55905, USA
    Gastroenterology 125:1364-9. 2003
    The epidemiology of primary sclerosing cholangitis (PSC) in the United States is unknown. We report the incidence, clinical spectrum, and outcomes of PSC in Olmsted County, Minnesota.
  21. ncbi Ursodeoxycholic acid aggravates bile infarcts in bile duct-ligated and Mdr2 knockout mice via disruption of cholangioles
    Peter Fickert
    Department of Medicine, Karl Franzens University, Graz, Austria
    Gastroenterology 123:1238-51. 2002
    ..The effects of ursodeoxycholic acid (UDCA) in biliary obstruction are unclear. We aimed to determine the effects of UDCA in bile duct-ligated and in Mdr2 knockout (Mdr2(-/-)) mice with biliary strictures...
  22. ncbi Genome-wide association analysis in primary sclerosing cholangitis
    Tom H Karlsen
    Medical Department, Rikshospitalet University Hospital and University of Oslo, 0027 Oslo, Norway
    Gastroenterology 138:1102-11. 2010
    We aimed to characterize the genetic susceptibility to primary sclerosing cholangitis (PSC) by means of a genome-wide association analysis of single nucleotide polymorphism (SNP) markers.
  23. ncbi Liver transplantation for primary sclerosing cholangitis in the Nordic countries: outcome after acceptance to the waiting list
    Bjørn Brandsaeter
    Department of Medicine, Rikshospitalet, Oslo, Norway
    Liver Transpl 9:961-9. 2003
    Primary sclerosing cholangitis (PSC) is a common indication for liver transplantation, but evaluation of patients and timing of liver transplantation remain as major problems...
  24. ncbi Latest and emerging therapies for primary biliary cirrhosis and primary sclerosing cholangitis
    Claudia O Zein
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Curr Gastroenterol Rep 12:13-22. 2010
    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults...
  25. ncbi Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis
    Kelly Burak
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Am J Gastroenterol 99:523-6. 2004
    Cholangiocarcinoma (CCA) is a dreaded complication of primary sclerosing cholangitis (PSC); however, marked variability in the incidence of CCA in PSC is reported...
  26. ncbi De-novo cholangiocarcinoma in native common bile duct remnant following OLT for primary sclerosing cholangitis
    Carmen Landaverde
    University of California, Los Angeles, Department of Medicine, Los Angeles, CA, USA
    Ann Hepatol 8:379-83. 2009
    Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory and obstructive disease of the intra- and extra-hepatic bile ducts of unknown etiology...
  27. ncbi Analysis of regulatory T cells and IgG4-positive plasma cells among patients of IgG4-related sclerosing cholangitis and autoimmune liver diseases
    Masanori Koyabu
    Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, 10 15 Fumizono, Moriguchi, Osaka, 570 8507, Japan
    J Gastroenterol 45:732-41. 2010
    ..The most common involved organ showing extrapancreatic lesions is the bile duct, which exhibits sclerosing cholangitis (SC). However, the role of IgG4 in the development of IgG4-related SC (IgG4-SC) remains unclear...
  28. pmc Lack of glycoprotein 130/signal transducer and activator of transcription 3-mediated signaling in hepatocytes enhances chronic liver injury and fibrosis progression in a model of sclerosing cholangitis
    Werner Plum
    Department of Medicine III, University Hospital Aachen, Aachen, Germany
    Am J Pathol 176:2236-46. 2010
    ..DDC) model leads to chronic cholestatic liver injury and therefore resembles human diseases such as sclerosing cholangitis and forms of metabolic liver diseases...
  29. ncbi High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis
    Rolf Olsson
    Department of Medicine, Sahlgrenska University Hospital Sahlgrenska, Goteborg, Sweden
    Eur J Intern Med 20:190-6. 2009
    Overlap syndrome is a term used for overlapping features of autoimmune hepatitis and primary sclerosing cholangitis or primary biliary cirrhosis and for autoimmune cholangitis...
  30. pmc The predictors of the presence of varices in patients with primary sclerosing cholangitis
    Sombat Treeprasertsuk
    Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, Rochester, MN, USA
    Hepatology 51:1302-10. 2010
    The predictors for developing varices in patients with primary sclerosing cholangitis (PSC) have not been well studied prospectively...
  31. pmc Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population
    C Y Ponsioen
    Department of Gastroenterology and Hepatology, Academic Medical Centre, Amsterdam, The Netherlands
    Gut 51:562-6. 2002
    Median survival of patients with primary sclerosing cholangitis (PSC) has been estimated to be 12 years. Cholangiography is the gold standard for diagnosis but is rarely used in estimating prognosis.
  32. ncbi Diagnostic accuracy of magnetic resonance and endoscopic retrograde cholangiography in primary sclerosing cholangitis
    Audun Elnaes Berstad
    Department of Radiology, Section of Gastroenterology, Rikshospitalet University Hospital, Oslo, Norway
    Clin Gastroenterol Hepatol 4:514-20. 2006
    To evaluate the accuracy and interobserver variability of magnetic resonance cholangiography (MRC) and endoscopic retrograde cholangiography (ERC) in the diagnosis of primary sclerosing cholangitis (PSC).
  33. ncbi Immunoglobulin G4-related sclerosing cholangitis in patients resected for presumed malignant bile duct strictures
    D Erdogan
    Department of Surgery, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
    Br J Surg 95:727-34. 2008
    ..The aim of this study was to assess the occurrence of this entity in benign bile duct strictures in patients resected for presumed hilar cholangiocarcinoma...
  34. pmc Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders
    S Engler
    Department of Medicine, Division of Gastroenterology, Hepatology and Infectious Diseases, University of Heidelberg, Germany
    Gut 52:688-93. 2003
    We present nine patients with progressive sclerosing cholangitis after septic shock.
  35. ncbi The natural history of small-duct primary sclerosing cholangitis
    Einar Bjornsson
    Department of Internal Medicine, Section of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Gothenburg, Sweden
    Gastroenterology 134:975-80. 2008
    The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized...
  36. ncbi Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis
    T Nakazawa
    First Department of Internal Medicine, Nagoya City University Medical School, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan
    Hepatogastroenterology 48:625-30. 2001
    BACKGROUND/AIMS: We encountered one group of primary sclerosing cholangitis cases which met classic criteria, but demonstrated better clinical courses...
  37. ncbi Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?
    Hideaki Hamano
    Department of Medicine, Shinshu University School of Medicine, Matsumoto 390 8621, Japan
    Gastrointest Endosc 62:152-7. 2005
    ..We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas...
  38. ncbi Primary sclerosing cholangitis and extraintestinal manifestations in patients with ulcerative colitis and ileal pouch-anal anastomosis
    Hans H Wasmuth
    Department of Gastrointestinal Surgery, St Olavs Hospital, Trondheim University Hospital, Trondheim, Norway
    J Gastrointest Surg 14:1099-104. 2010
    ..outcomes in patients having ileal pouch-anal anastomosis for ulcerative colitis with or without primary sclerosing cholangitis or extraintestinal manifestations and to assess if primary sclerosing cholangitis is a risk factor for ..
  39. ncbi Autoimmune hepatitis and primary sclerosing cholangitis
    Fernando Alvarez
    Department of Pediatrics, Hopital Sainte Justine, University of Montreal, 3175 Cote Sainte Catherine, Montreal, Quebec H3T 1C5, Canada
    Clin Liver Dis 10:89-107, vi. 2006
    Autoimmune liver disease in children presents predominantly as autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC)...
  40. ncbi Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for a metaplasia-dysplasia-carcinoma sequence
    Jason T Lewis
    Division of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905, USA
    Am J Surg Pathol 31:907-13. 2007
    Primary sclerosing cholangitis (PSC) carries an increased risk (10% to 20%) of hepatobiliary malignancy, especially cholangiocarcinoma (CC)...
  41. ncbi Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up
    C Jaeger
    Department of Medicine II, HSK Wiesbaden Teaching Hospital of the University of Mainz, Wiesbaden, Germany
    Endoscopy 38:730-4. 2006
    We present ten patients who developed secondary sclerosing cholangitis following long-term treatment in an intensive care unit (ICU) between 1999 and 2004.
  42. ncbi Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatment
    Daniel N Gotthardt
    Department of Medicine, University of Heidelberg, Heidelberg, Germany
    Gastrointest Endosc 71:527-34. 2010
    Primary sclerosing cholangitis is characterized by progressive fibrotic inflammation and obliteration of intra- and/or extrahepatic bile ducts. Total or subtotal stenoses of major bile ducts are associated with reduced survival.
  43. ncbi Diagnosis of primary sclerosing cholangitis: a blinded comparative study using magnetic resonance cholangiography and endoscopic retrograde cholangiography
    Stephen L Moff
    Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Gastrointest Endosc 64:219-23. 2006
    We hypothesized that magnetic resonance cholangiography (MRC) may have less accuracy for the diagnosis and the assessment of the severity of primary sclerosing cholangitis (PSC) than endoscopic retrograde cholangiography (ERC).
  44. ncbi Evolution of autoimmune hepatitis to primary sclerosing cholangitis: A sequential syndrome
    Ayman A Abdo
    Liver Unit, Division of Gastroenterology, University of Calgary, Calgary, Alberta, Canada
    Hepatology 36:1393-9. 2002
    Recently, the autoimmune hepatitis (AIH)/primary sclerosing cholangitis (PSC) overlap syndrome has been reported increasingly. In this syndrome, patients present with features of both AIH and PSC...
  45. ncbi Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis
    M Oswald
    Department of Medicine II, Klinikum Grosshadern, University of Munich, 81377 Munich, Germany
    Liver 21:247-53. 2001
    ..SLC21A6) and the canalicular multidrug resistance protein 2 (MRP2) was studied in patients with primary sclerosing cholangitis (PSC) (n=4), a chronic cholestatic liver disease, and in non-cholestatic controls (n=4) (two with chronic ..
  46. ncbi Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden
    Bjorn Lindkvist
    Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
    Hepatology 52:571-7. 2010
    Population-based studies on the epidemiology of primary sclerosing cholangitis (PSC) are sparse...
  47. pmc Cigarette smoking, appendectomy, and tonsillectomy as risk factors for the development of primary sclerosing cholangitis: a case control study
    S A Mitchell
    Department of Gastroenterology, Oxford Radcliffe Hospital, Oxford, UK
    Gut 51:567-73. 2002
    The strong clinical association between primary sclerosing cholangitis (PSC) and ulcerative colitis (UC) suggests common factors in their pathogenesis...
  48. ncbi IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material
    Vikram Deshpande
    The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA
    Mod Pathol 22:1287-95. 2009
    ..condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained ..
  49. pmc A new xenobiotic-induced mouse model of sclerosing cholangitis and biliary fibrosis
    Peter Fickert
    Laboratory of Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Medicine, Medical University Graz, Austria
    Am J Pathol 171:525-36. 2007
    ..This model may be valuable to investigate the mechanisms of xenobiotic-induced chronic cholangiopathies and its sequels including biliary fibrosis...
  50. pmc The immunobiology of primary sclerosing cholangitis
    Jonathan H Aron
    Department of Internal Medicine, University of California Davis Medical Center, 4150 V Street, PSSB 3100, Sacramento, CA 95817, USA
    Semin Immunopathol 31:383-97. 2009
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease histologically characterized by the presence of intrahepatic and/or extrahepatic biliary duct concentric, obliterative fibrosis, eventually leading to cirrhosis...
  51. pmc Curcumin improves sclerosing cholangitis in Mdr2-/- mice by inhibition of cholangiocyte inflammatory response and portal myofibroblast proliferation
    Anna Baghdasaryan
    Laboratory of Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University Graz, Graz, Austria
    Gut 59:521-30. 2010
    ..Curcumin, the yellow pigment of the spice turmeric, has pleiotropic actions and attenuates hepatic damage in animal models of chemically-induced liver injury. Whether curcumin has beneficial effects in cholangiopathies is unknown...
  52. ncbi Update on primary sclerosing cholangitis
    Tom H Karlsen
    Norwegian PSC Research Center, Medical Department, Oslo University Hospital, Rikshospitalet, Oslo, Norway
    Dig Liver Dis 42:390-400. 2010
    Early studies in primary sclerosing cholangitis (PSC) were concerned with disease characterization, and were followed by epidemiological studies of PSC and clinical subsets of PSC as well as a large number of treatment trials...
  53. ncbi Sclerosing cholangitis: a focus on secondary causes
    Rupert Abdalian
    Department of Medicine, University Health Network, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada
    Hepatology 44:1063-74. 2006
    Secondary sclerosing cholangitis (SSC) is a disease that is morphologically similar to primary sclerosing cholangitis (PSC) but that originates from a known pathological process...
  54. ncbi High serum IgG4 concentrations in patients with sclerosing pancreatitis
    H Hamano
    Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan
    N Engl J Med 344:732-8. 2001
    ..and 154 patients with pancreatic cancer, ordinary chronic pancreatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or Sjögren's syndrome...
  55. ncbi Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis
    Roy M Soetikno
    Gastroenterology Section, VA Palo Alto and Department of Medicine, Division of Gastroenterology, Stanford University School of Medicine, Palo Alto, California 94304, USA
    Gastrointest Endosc 56:48-54. 2002
    ..data on the risk of colorectal neoplasia in patients with ulcerative colitis with and without primary sclerosing cholangitis are conflicting...
  56. pmc Identification of novel molecules and pathogenic pathways in primary biliary cirrhosis: cDNA array analysis of intrahepatic differential gene expression
    N A Shackel
    AW Morrow Gastroenterology and Liver Centre, Centenary Institute of Cancer Medicine and Cell Biology, Royal Prince Alfred Hospital and the University of Sydney, Sydney, Australia
    Gut 49:565-76. 2001
    ..METHODS: Utilising cDNA arrays of 874 genes, PBC was compared with primary sclerosing cholangitis (PSC) associated cirrhosis and non-diseased liver...
  57. ncbi 24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice
    Peter Fickert
    Department of Medicine, Laboratory of Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Medical University Graz, Austria
    Gastroenterology 130:465-81. 2006
    Current therapy for primary sclerosing cholangitis is of limited efficacy. Multidrug resistance gene 2 knockout mice (Mdr2(-/-)) represent a well-characterized model for sclerosing cholangitis...
  58. ncbi Particular genetic variants of ligands for natural killer cell receptors may contribute to the HLA associated risk of primary sclerosing cholangitis
    Tom H Karlsen
    Medical Department, Rikshospitalet Radiumhospitalet Medical Center, University of Oslo, Oslo, Norway
    J Hepatol 46:899-906. 2007
    ..We aimed to clarify to what extent such combinations influence susceptibility to primary sclerosing cholangitis (PSC).
  59. ncbi Different HLA class II associations in ulcerative colitis patients with and without primary sclerosing cholangitis
    T H Karlsen
    Medical Department, Rikshospitalet Radiumhospitalet Medical Center, University of Oslo, Oslo, Norway
    Genes Immun 8:275-8. 2007
    Approximately 80% of patients with primary sclerosing cholangitis (PSC) of Northern European origin have inflammatory bowel disease (IBD), the majority ulcerative colitis (UC)...
  60. ncbi Biliary epithelial cell antibodies link adaptive and innate immune responses in primary sclerosing cholangitis
    Azza Karrar
    Division of Transplantation Surgery, Division of Clinical Immunology, Karolinska University Hospital Huddinge, Stockholm, Sweden
    Gastroenterology 132:1504-14. 2007
    Primary sclerosing cholangitis (PSC) is an autoimmune liver disease with destruction of hepatic bile ducts. A high frequency of biliary epithelial cell antibodies (BEC-Ab) is present in PSC...
  61. ncbi Small-duct primary sclerosing cholangitis: a long-term follow-up study
    Paul Angulo
    Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Hepatology 35:1494-500. 2002
    ..inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography...
  62. ncbi Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation
    K M Boberg
    Medical Dept, Rikshospitalet, Oslo, Norway
    Scand J Gastroenterol 37:1205-11. 2002
    Primary sclerosing cholangitis (PSC) confers a high risk of cholangiocarcinoma (CC) development. Since patients at risk of CC may be selected for early liver transplantation, it is a challenge to identify any predisposing factors...
  63. ncbi Probiotics in primary sclerosing cholangitis: a randomized placebo-controlled crossover pilot study
    Frank P Vleggaar
    Department of Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands
    Eur J Gastroenterol Hepatol 20:688-92. 2008
    ..Inflammatory bowel disease concurs in 90% of primary sclerosing cholangitis (PSC) patients, suggesting that substances originating from the inflamed gut may damage the biliary tree.
  64. ncbi Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome
    Annarosa Floreani
    Department of Surgical and Gastroenterological Sciences, University of Padova, Padova, Italy
    Am J Gastroenterol 100:1516-22. 2005
    Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC.
  65. ncbi Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis
    Takahiro Nakazawa
    Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
    Pancreas 30:20-5. 2005
    b>Sclerosing cholangitis (SC) with autoimmune pancreatitis (AIP) is similar to primary sclerosing cholangitis (PSC) with regard to cholangiographic findings, but only the former responds well to steroid therapy...
  66. ncbi Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population
    T M Neri
    Department of Internal Medicine, Nephrology and Health Sciences, University of Parma, Parma, Italy
    Dig Liver Dis 35:571-6. 2003
    In patients with with primary sclerosing cholangitis we investigated the major histocompatibility complex (MHC) genes and mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
  67. ncbi Mapping MHC-encoded susceptibility and resistance in primary sclerosing cholangitis: the role of MICA polymorphism
    S Norris
    Guy's, King's and Thomas' (GKT) Combined Medical Schools, London, England
    Gastroenterology 120:1475-82. 2001
    ..amp; AIMS: Recent studies suggest that major histocompatibility complex-encoded susceptibility to primary sclerosing cholangitis (PSC) maps to the HLA B-TNFA region on chromosome 6p21.3...
  68. ncbi Chemotherapy-induced sclerosing cholangitis: long-term response to endoscopic therapy
    Waleed M Alazmi
    Indiana University Medical Center, 550 N University Blvd, Indianapolis, 46202, USA
    J Clin Gastroenterol 40:353-7. 2006
    ..One major complication of such treatment is biliary sclerosis resembling primary sclerosing cholangitis, which has an incidence ranging from 8% to 26%.
  69. ncbi Primary sclerosing cholangitis: outcome of patients undergoing restorative proctocolecetomy for ulcerative colitis
    Anna Lepistö
    Department of Surgery, Helsinki University Central Hospital, PL 340, HUS, 00029, Helsinki, Finland
    Int J Colorectal Dis 24:1169-74. 2009
    The prevalence of primary sclerosing cholangitis (PSC) among patients with ulcerative colitis needing proctocolectomy is about 12%. The study aim was to evaluate the progression of the liver disease after surgery.
  70. ncbi Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis
    Takayoshi Nishino
    Institute of Gastroenterology, Department of Medicine, Tokyo Women s Medical University, School of Medicine, 8 1 Kawada cho, Shinjuku ku, Tokyo 162 8666, Japan
    J Gastroenterol 42:550-9. 2007
    The present study was undertaken to identify the clinicopathological differences between sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and primary sclerosing cholangitis (PSC).
  71. ncbi Systemic phaeohyphomycosis resembling primary sclerosing cholangitis caused by Exophiala dermatitidis
    Erkin Oztas
    Department of Gastroenterology, Turkiye Yuksek Ihtisas Teaching and Research Hospital, Ankara, Turkey
    J Med Microbiol 58:1243-6. 2009
    ..We report a case of systemic phaeohyphomycosis resembling sclerosing cholangitis caused by E. dermatitidis in a 24-year-old woman.
  72. ncbi Azithromycin may reduce cholestasis in primary sclerosing cholangitis: a case report and serendipitous observation
    A L Boner
    Department of Pediatrics, University of Verona, Italy
    Int J Immunopathol Pharmacol 20:847-9. 2007
    ..We report the possible effect of azithromycin on primary sclerosing cholangitis in a patient treated with the drug for severe asthma...
  73. ncbi Sclerosing cholangitis caused by old shrapnel injury
    William B Silverman
    Division of Gastroenterology and Hepatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
    Gastrointest Endosc 58:420. 2003
  74. ncbi Long-term outcomes of positive fluorescence in situ hybridization tests in primary sclerosing cholangitis
    Sanjay Y Bangarulingam
    Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USA
    Hepatology 51:174-80. 2010
    Patients with primary sclerosing cholangitis (PSC) are at increased risk for developing cholangiocarcinoma (CCA). Fluorescence in situ hybridization (FISH) is a cytological test designed to enhance early CCA diagnosis...
  75. ncbi Outcome following liver transplantation for primary sclerosing cholangitis in the Nordic countries
    B Brandsaeter
    Dept of Medicine, Rikshospitalet, Oslo, Norway
    Scand J Gastroenterol 38:1176-83. 2003
    Primary sclerosing cholangitis (PSC) is the most common indication for liver transplantation in the Nordic countries...
  76. ncbi Liver transplantation for primary sclerosing cholangitis: timing, outcome, impact of inflammatory bowel disease and recurrence of disease
    R H Wiesner
    Liver Transplant Center, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Best Pract Res Clin Gastroenterol 15:667-80. 2001
    Over the past decade, the outcome of liver transplantation in primary sclerosing cholangitis (PSC) patients with end-stage liver disease has improved significantly with many centres reporting 1-year patient and graft survival of 90-97% ..
  77. ncbi Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation
    Jean Charles Duclos-Vallée
    Centre Hepato Biliaire, AP HP Hopital Paul Brousse, Villejuif, France
    Liver Transpl 15:S25-34. 2009
    1. Recurrence of primary sclerosing cholangitis. a Five percent of liver transplants are performed because of end-stage liver disease secondary to primary sclerosing cholangitis. b Recurrent disease affects 10% to 27% of recipients...
  78. ncbi Fatigue in patients with primary sclerosing cholangitis
    E Bjornsson
    Department of Internal Medicine, Section of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Gothenburg, Sweden
    Scand J Gastroenterol 39:961-8. 2004
    The occurrence of fatigue in primary sclerosing cholangitis (PSC), its impact on quality of life and the role of concomitant inflammatory bowel disease (IBD) and coexisting irritable bowel syndrome (IBS) is unexplored.
  79. ncbi Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy
    B Brandsaeter
    Section of Hepatology and Gastroentrology, Department of Medicine, Rikshospitalet, 0027 Oslo, Norway
    J Hepatol 40:815-22. 2004
    Hepatobiliary malignancies are frequently seen in primary sclerosing cholangitis (PSC) and they complicate the evaluation of patients and timing of liver transplantation.
  80. ncbi Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature
    Einar Bjornsson
    Department of Internal Medicine, Division of Gastroenterology and Hepatology, Sahlgrenska University Hospital, SE 413 45 Gothenburg, Sweden
    Hepatology 45:1547-54. 2007
  81. ncbi Secondary sclerosing cholangitis: a comparison to primary sclerosing cholangitis
    Andrea A Gossard
    Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Am J Gastroenterol 100:1330-3. 2005
    The natural history of secondary sclerosing cholangitis (SSC) is ill-defined. In order to better determine the natural history of this condition, we retrospectively reviewed data from the Mayo Clinic in Rochester, Minnesota...
  82. ncbi Medical treatment of primary sclerosing cholangitis: a role for novel bile acids and other (post-)transcriptional modulators?
    Ulrich Beuers
    Department of Gastroenterology and Hepatology, G4 213, Academic Medical Center, University of Amsterdam, P O Box 22700, 1100, DE, Amsterdam, The Netherlands
    Clin Rev Allergy Immunol 36:52-61. 2009
    Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic disease of the liver and bile ducts that is associated with inflammatory bowel disease, generally leads to end-stage liver disease, and is complicated by malignancies of ..
  83. ncbi Concurrent oral lichen planus and primary sclerosing cholangitis
    D C Tong
    Department of Stomatology, University of Otago, PO Box 647, Dunedin, New Zealand
    Br J Dermatol 147:356-8. 2002
    Four patients with concomitant oral lichen planus (OLP) and primary sclerosing cholangitis (PSC) are presented...
  84. pmc Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis
    Terumi Kamisawa
    Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3 18 22 Honkomagome, Bunkyo ku, Tokyo, Japan
    World J Gastroenterol 15:2357-60. 2009
    To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC).
  85. pmc Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: Relation to disease progression
    Emmanouil Sinakos
    Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USA
    Hepatology 52:197-203. 2010
    ..mg/kg/day) ursodeoxycholic acid (UDCA) treatment improves serum liver tests in patients with primary sclerosing cholangitis (PSC) but does not improve survival and is associated with increased rates of serious adverse events...
  86. ncbi Pretransplant prediction of prognosis after liver transplantation in primary sclerosing cholangitis using a Cox regression model
    J Neuberger
    The Liver and Hepatibiliary Unit, The Queen Elizabeth Hospital, Edgbaston, Birmingham, UK
    Hepatology 29:1375-9. 1999
    Liver transplantation remains the only treatment for patients with end-stage primary sclerosing cholangitis (PSC); however, selection criteria for the procedure and its timing remains uncertain...
  87. ncbi Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis
    Gerda Rudolph
    Department of Medicine, University of Heidelberg, Medizinische Universitatsklinik, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany
    J Hepatol 51:149-55. 2009
    In primary sclerosing cholangitis (PSC) dominant stenoses are frequently associated with bacterial, and in part, also fungal infections of the bile ducts...
  88. ncbi Detecting cholangiocarcinoma in patients with primary sclerosing cholangitis
    Eduardo Siqueira
    Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
    Gastrointest Endosc 56:40-7. 2002
    Primary sclerosing cholangitis is a progressive cholestatic liver disease associated with cholangiocarcinoma...
  89. ncbi Systemic lupus erythematosus with concurrent protein-losing enteropathy and primary sclerosing cholangitis: a unique association
    D C T Oh
    Division of Gastroenterology, Department of Medicine, Changi General Hospital, Singapore
    Lupus 15:102-4. 2006
    ..SLE) with the gastrointestinal manifestations of protein-losing enteropathy (PLE) and primary sclerosing cholangitis (PSC). He presented with periorbital, scrotal and lower limb oedema...
  90. ncbi Therapeutic benefit of sulfasalazine for patients with primary sclerosing cholangitis
    Shinichiro Tada
    J Gastroenterol 41:388-9. 2006
  91. ncbi Pathologic features of ulcerative colitis in patients with primary sclerosing cholangitis: a case-control study
    Mee Joo
    Inje University Ilsan Paik Hospital, Goyang, Korea
    Am J Surg Pathol 33:854-62. 2009
    The pathologic features of ulcerative colitis (UC) in patients with primary sclerosing cholangitis (PSC) are, essentially, unknown...
  92. ncbi Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis
    Hyoung Chul Oh
    Department of Internal Medicine, Chung Ang University College of Medicine, Seoul, Korea
    Intern Med 47:2025-8. 2008
    ..and cholestasis are among bile duct cancer risk factors, the cause-and-effect relationship between sclerosing cholangitis and bile duct cancer needs to be determined by further study, as between AIP and pancreatic cancer...
  93. ncbi A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts
    Edward Alabraba
    Liver Unit, University Hospital Birmingham NHS Foundation Trust, Birmingham, United Kingdom
    Liver Transpl 15:330-40. 2009
    ..that the absence of the colon after liver transplantation (LT) protects the patient from recurrent primary sclerosing cholangitis (rPSC)...
  94. ncbi Thyroid dysfunction in primary biliary cirrhosis, primary sclerosing cholangitis and non-alcoholic fatty liver disease
    Marina G Silveira
    Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USA
    Liver Int 29:1094-100. 2009
    ..Primary biliary cirrhosis (PBC) is frequently associated with autoimmune diseases, including thyroid disease, although it is uncertain that this association is higher than in other liver diseases...
  95. ncbi Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis
    Laura E Moreno Luna
    Division of Gastroenterology and Hepatology, Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    Liver Transpl 12:S15-9. 2006
  96. ncbi Co-existence of primary biliary cirrhosis and primary sclerosing cholangitis: a rare overlap syndrome put in perspective
    Jeremy G C Kingham
    Department of Gastroenterology, Singleton Hospital, Swansea, UK
    Eur J Gastroenterol Hepatol 17:1077-80. 2005
    Overlap syndromes between autoimmune hepatitis and both primary biliary cirrhosis and primary sclerosing cholangitis are well described, but overlap between primary biliary cirrhosis and primary sclerosing cholangitis is not recognized...
  97. ncbi Unique features of primary sclerosing cholangitis in children
    Giorgina Mieli-Vergani
    Paediatric Liver Centre, Institute of Liver Studies, King s College London School of Medicine at King s College Hospital, London, UK
    Curr Opin Gastroenterol 26:265-8. 2010
    To summarize publications on juvenile primary sclerosing cholangitis (PSC) published over the past 5 years...
  98. ncbi Characterization and clinical course of hepatobiliary carcinoma in patients with primary sclerosing cholangitis
    Jens J W Tischendorf
    Department of Gastroenterology, Hepatology and Endocrinology, Medical School of Hannover, Germany
    Scand J Gastroenterol 41:1227-34. 2006
    Primary sclerosing cholangitis (PSC) confers a high risk of development of hepatobiliary carcinoma (HBC). The aim of the study was to identify indicators and risk factors for developing HBC in PSC patients.
  99. ncbi Poor outcome in progressive sclerosing cholangitis after septic shock
    H Kulaksiz
    Department of Internal Medicine, Division of Gastroenterology, University Hospital Ulm, Germany
    Endoscopy 40:214-8. 2008
    Progressive sclerosing cholangitis after septic shock is an increasingly diagnosed disease entity. We evaluated the outcome after long-term follow-up of 29 patients treated in our institution between 1995 and 2007.
  100. ncbi Sclerosing cholangitis-like changes in hepatobiliary tuberculosis
    Yasemin Ozin
    Department of Gastroenterology, Turkiye Yuksek Ihtisas Training and Research Hospital, Ankara, Turkey
    Turk J Gastroenterol 21:50-3. 2010
    ..irregularities, strictures and dilatations both in the intra- and extrahepatic bile ducts resembling sclerosing cholangitis. The liver biopsy showed caseous granulomatous hepatitis and Langhans giant cells compatible with ..
  101. ncbi Small-duct primary sclerosing cholangitis. A single-center seven-year experience
    Nikolaos L Nikolaidis
    Gastroenterology and Hepatology Section, Second Propedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, 54642 Greece
    Dig Dis Sci 50:324-6. 2005
    Patients with cholestatic liver function tests and histological features of primary sclerosing cholangitis (PSC) but without the typical cholangiographic changes are considered to have small-duct PSC...

Research Grants60

  1. Histamine modulation of biliary proliferation and damage
    Heather L Francis; Fiscal Year: 2013
    ..applicant): Cholangiocytes are key target cells in diseases such as primary biliary cirrhosis and primary sclerosing cholangitis collectively known as cholangiopathies...
  2. Liver disease in CF: CFTR controls innate immunity in biliary epithelium
    Mario Strazzabosco; Fiscal Year: 2013
    ..and hepatobiliary complications characterized by a chronic cholangiopathy that can eventually evolve into sclerosing cholangitis and focal biliary cirrhosis...
  3. GROWTH REGULATION OF THE INTRAHEPATIC BILIARY TREE
    GIANFRANCO D ALPINI; Fiscal Year: 2010
    ..during chronic cholestatic liver diseases (cholangiopathies) such as primary biliary cirrhosis, primary sclerosing cholangitis, and cholangiocarcinoma...
  4. Role of the Renin-Angiotensin System in the Regulation of Biliary Proliferation
    SHANNON STROUD GLASER; Fiscal Year: 2010
    ..are lacking for chronic cholestatic liver diseases, such as primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). Chronic cholestatic liver diseases cause damage to the bile ducts of the liver...
  5. Developing Novel Serological Biomarkers for Autoimmune Liver Diseases
    Xuhang Li; Fiscal Year: 2013
    ..liver diseases (AILDs), including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), are chronic and incurable immune disorders of the liver...
  6. Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing CholangitisA pilot
    Benjamin L Shneider; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Primary sclerosing cholangitis (PSC), a devastating and insidiously progressive cholestatic liver disease, results from advancing inflammation, fibrosis and obliteration of the intra- and extrahepatic ..
  7. Novel prognostic microRNA biomarkers for primary sclerosing cholangitis
    Kris Kowdley; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Primary sclerosing cholangitis (PSC) is a progressive chronic cholestatic liver disease of unknown etiology...
  8. Access to Liver Transplantation for Patients with Primary Sclerosing Cholangitis
    David Seth Goldberg; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Primary sclerosing cholangitis (PSC) is a chronic liver disease that has no treatment and is an otherwise fatal disease without liver transplantation...
  9. Dysregulated Hypothalamic-pituitary-adrenal Axis During Biliary Hyperplasia
    Sharon Demorrow; Fiscal Year: 2013
    ..provided by applicant): Cholestatic liver diseases such as primary biliary cirrhosis and primary sclerosing cholangitis are often associated with increased serum bile acid concentrations...
  10. Regulation of Bile Duct Growth in Bile Duct Ligated Rats
    SHANNON STROUD GLASER; Fiscal Year: 2013
    ..are the target cells in cholestatic liver diseases such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), which are characterized by the damage and proliferation of these cells...
  11. Role of Sensory Neuropeptides in the Regulation of Biliary Function
    Fanyin Meng; Fiscal Year: 2013
    ..during the pathogenesis of cholestatic liver diseases such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)...
  12. MicroRNA involvement in the regulation of biliary epithelium
    Florin Selaru; Fiscal Year: 2013
    ..I am hopeful that through the findings of this project, we will make significant progress towards understanding how and why inflammatory conditions of the biliary tree, such as Primary Sclerosing Cholangitis, progress to neoplasia.
  13. Pathophysiology of Biliary Disease
    Nicholas F Larusso; Fiscal Year: 2013
    ..mechanisms, consequences and pathologic outcomes of cholangiocyte senescence in the syndrome of primary sclerosing cholangitis (PSC)...
  14. Organelle Dysfunction and Apoptosis in Liver Epithelia
    Gregory J Gores; Fiscal Year: 2013
    ..g., primary sclerosing cholangitis)...
  15. PSC Resource Of Genetic Risk, Environment and Synergy Studies (PROGRESS)
    Konstantinos N Lazaridis; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Primary sclerosing cholangitis (PSC) is a progressive liver disease strongly associated with inflammatory bowel disease (IBD)...
  16. The role of CDK5 in cholangiocyte differentiation and morphogenesis
    JUSTIN MICHAEL NUSSBAUM; Fiscal Year: 2012
    ..g., primary biliary cirrhosis and primary sclerosing cholangitis) are a major cause of chronic liver disease...
  17. CATALASE IMMUNITY IN PRIMARY SCLEROSING CHOLANGITIS
    James Versalovic; Fiscal Year: 2005
    DESCRIPTION (adapted from the application) Primary sclerosing cholangitis (PSC) includes features of autoimmunity...
  18. METHODOLOGIC RESEARCH IN SIMULATING COMPLEX DISEASE
    Mark Roberts; Fiscal Year: 1991
    ..evaluation of liver transplantation recommends transplantation for ESLD from primary biliary cirrhosis or sclerosing cholangitis. However, the timing of liver transplantation is an important clinical question that has received little ..
  19. PILOT STUDY OF PIRFENIDONE IN PBC AND PSC
    Keith Lindor; Fiscal Year: 1999
    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are progressive chronic cholestatic liver diseases...
  20. BILIARY CALMODULIN DEPENDENT PROTEIN KINASE II ISOFORMS
    ANN STANSFIELD; Fiscal Year: 2000
    ..In turn, this suggests that other cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing Cholangitis, sarcoidosis, liver transplant rejection and AIDS Cholangiopathy may also occur from or cause alterations ..
  21. Aberrant kinase signaling in cholangiocarcinoma
    Tushar Patel; Fiscal Year: 2003
    ..Chronic inflammation of the biliary tract such as in primary sclerosing cholangitis or hepatolithiasis is characterized by alterations in the cytokine milieu and predisposes to the ..
  22. Gene delivery to bile duct epithelial cells in mice
    Vladimir Subbotin; Fiscal Year: 2001
    Primary biliary cirrhosis and primary sclerosing cholangitis are irreversible liver diseases of unknown etiology with no available treatment or preventive therapy, and only liver transplantation can prolong life of patients with disease ..
  23. Mechanisms of Carcinogenesis in Biliary Epithelia
    Gregory Gores; Fiscal Year: 2006
    ..preliminary data demonstrating that biliary epithelia in a pre-malignant inflammatory disease (primary sclerosing cholangitis), and even cholangiocarcinomas paradoxically express the potent apoptosis inducing ligand TRAIL (tumor ..
  24. DYSREGULATION OF CHOLANGIOCYTE APOPTOSIS BY CYTOKINES
    Tushar Patel; Fiscal Year: 2003
    ..Primary sclerosing cholangitis, a chronic inflammatory condition affecting the biliary system, is associated with cytokine alterations ..
  25. CD40-CD154 Interactions in Cryptosporidial Immunity
    ESTHER PONNURAJ; Fiscal Year: 2001
    ..AIDS or mutated CD154 genes (X linked immunodeficiency with hyper IgM, or XHIM) with the development of sclerosing cholangitis and a need for liver transplant...
  26. CD40-CD154 Interactions in Cryptosporidial Immunity
    ESTHER PONNURAJ; Fiscal Year: 2004
    ..in humans with AIDS or mutated CD154 genes (X linked immunodeficiency with hyper IgM, or XHIM) leading to sclerosing cholangitis and liver failure...
  27. PROTECTION OF HOST TISSUES FROM FDURD TOXICITY
    Jonathan Maybaum; Fiscal Year: 2002
    ..for the treatment of intrahepatic neoplasms, the dose-limiting toxicity is biliary sclerosis (also called sclerosing cholangitis)...
  28. TCR USAGE AND FUNCTION IN IBD
    Lawrence Saubermann; Fiscal Year: 2003
    ..usage of the bile-duct associated T-cells in the clinically distinct subset of individuals with primary sclerosing cholangitis, and; (3) the relationship between major histocompatibility complex (MHC) and TCR usage in families with ..
  29. Oxidative mutagenesis:Iron, Ascorbic Acid and Genes
    Christopher Bowlus; Fiscal Year: 2002
    ..They will use a mouse model of HH that is prone to hepatocellular formation. ..
  30. TFF3 GENE EXPRESSION IN METASTATIC COLON CANCER
    Steven Itzkowitz; Fiscal Year: 2001
    ..The use of the TFF3 gene to drive expression of suicide genes offers a novel approach which should result in more targetted, safer gene therapy for metastatic colon cancer. ..
  31. 1Treatment of Cholestatic Pruritus With Sertraline
    MARLYN MAYO; Fiscal Year: 2004
    ..These studies will serve as pilot trials. The results of this proposed project will be used to design a larger controlled trial that could be proposed for funding through an R01 mechanism. ..
  32. ROLE OF CD40-CD40 LIGAND IN PRIMARY BILIARY CIRRHOSIS
    MARLYN MAYO; Fiscal Year: 2003
    ..These studies will provide insight into the role of T cells in the pathogenesis of PBC and provide information useful in developing new treatment modalities. ..
  33. Clinical Approaches to Ileal Pouch Dysfunction
    Bo Shen; Fiscal Year: 2005
    ..The AIMS of study are to 1) investigate visceral hypersensitivity using barostat examination of the pouch; 2) conduct a randomized, placebo-controlled clinical trial evaluating the use of amitriptyline in IPS. ..
  34. Trefoil Factors in GI Cancer Biology
    Steven H Itzkowitz; Fiscal Year: 2010
    ..The results of these studies could provide insights into novel treatment strategies for gastrointestinal malignancies. ..
  35. Longitudinal Studies of Primary Biliary Cirrhosis
    MARLYN MAYO; Fiscal Year: 2006
    ..In addition, the correlation between each of the putative surrogate markers will be analyzed. The results of this analysis will then be used to design future clinical trials in the field of PBC. [unreadable] [unreadable]..
  36. BORAGE OIL AND GINKGO BILOBA (EGb 761) IN ASTHMA
    MERRILL GERSHWIN; Fiscal Year: 2004
    ..It is hoped that findings from these studies will evolve relatively non-toxic therapeutic alternatives for attenuating bronchial hyperresponsiveness and inflammation in patients with asthma. ..
  37. HCV Quasispecies Dynamics and Liver Transplant
    Hugo Vargas; Fiscal Year: 2005
    ..We will separate viral variants bound in immunocomplexes from "free" virus and compare the variants using SSCP, cloning and sequencing. ..
  38. PHENOTYPIC AND PSYCHOSOCIAL STUDY OF THE L1307K MUTATION
    Henry Lynch; Fiscal Year: 2004
    ....
  39. Molecular and Functional Analysis of CASK in Epithelia
    Zenta Walther; Fiscal Year: 2005
    ..We anticipate these studies will shed considerable light on the molecular interactions of CASK, its role in growth factor complex assembly and/or signal transduction, and its potential involvement in tumorigenesis. ..
  40. IDENTIFYING MAJOR SUSCEPTIBILITY GENES OF IBD
    JUDY CHO; Fiscal Year: 2003
    ..Once risk alleles are identified in multiplex families, characterization of the nature and broad significance of specific risk alleles for IBD in a variety of populations will be performed. ..
  41. MOLECULAR BASIS OF SECRETORY DIARRHEA
    Jonathan Cohn; Fiscal Year: 2003
    ..Given the pivotal role of CFTR in the pathogenesis of diarrhea and CF this project has excellent prospects of leading to information of practical benefit in the treatment of these conditions. ..
  42. HEPATITIS C VIRUS COINFECTION IN HIV-1 INFECTED SUBJECTS
    Jorge Rakela; Fiscal Year: 2002
    ..In summary, our studies will further characterize extrahepatic replication of HCV in HIV-1 infected subjects and will elucidate its role in mother-to-infant transmission of these two viruses. ..
  43. Ariol SL-50 Workstation and Review Stations
    MARY WASHINGTON; Fiscal Year: 2005
    ..An additional benefit is the ease of data sharing among groups, facilitating interactions among investigators and promoting inter-SPORE collaborations. ..
  44. The Role of GDF-15 in Organ Injury
    Leonidas Koniaris; Fiscal Year: 2005
    ..The completion of this mentored research training will further the candidate?s goal of applying bench-derived scientific discovery to the treatment of surgical disease as an independent investigator. ..
  45. Pathophysiological Interpretations of Dynamic PET/CT of Molecular Liver Biology
    Susanne Keiding; Fiscal Year: 2010
    ..This will lead to improved diagnosis and treatment of the large groups of patients with liver disease and cancer in the liver. ..
  46. Epidemiology and Impact of Hepatitis C in the Community
    W Ray Kim; Fiscal Year: 2007
    ..abstract_text> ..
  47. Increasing Functional Liver Mass in Health and Disease
    Leonidas Koniaris; Fiscal Year: 2005
    ..2) Determine the extent to which and mechanisms by which co-administrating HCF and Follistatin along with IL-6 facilitates the liver growth response and reduces IL-6 associated toxicity. ..
  48. HCV-HIV Coinfection: Impact of Immune Dysfunction
    Richard Sterling; Fiscal Year: 2007
    ..The K-23 award will protect this candidates time and help him mature into a senior investigator in Hepatology. ..
  49. Relationship of metabolic abnormalities to hepatic steatosis in HIV
    Richard Sterling; Fiscal Year: 2008
    ..If our hypothesis is correct, this work could result in a dramatic change in the long-term care of the over 1 million HIV infected individuals in the US. [unreadable] [unreadable]..
  50. Genetic Epidemiology of Primary Biliary Cirrhosis
    Konstantinos Lazaridis; Fiscal Year: 2008
    ..abstract_text> ..
  51. GM-CSF Enhanced Colorectal Cancer Tumor Vaccine Program
    Richard Schulick; Fiscal Year: 2008
    ..abstract_text> ..
  52. Mapping Chromosome 3q Inflammatory Bowel Disease Genes
    JUDY CHO; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  53. Dissecting the Aggregation of AMA in Families of PBC Patients
    Konstantinos Lazaridis; Fiscal Year: 2008
    ..If successful, our effort will shed light toward better understanding of the genetic predisposition to PBC. [unreadable] [unreadable]..
  54. Listeria Monocytogenes to Treat Hepatic Metastases
    Richard Schulick; Fiscal Year: 2009
    ..Specific Aim 4: Define the role of dendritic cells in the priming and targeting of the immune response. ..
  55. Lymphocyte recruitment in alcoholic hepatitis
    David Adams; Fiscal Year: 2009
    ..abstract_text> ..
  56. The Cystic Fibrosis Gene and Chronic Pancreatitis
    Jonathan Cohn; Fiscal Year: 2009
    ..Thus, this project's general goals are to develop methods to identify health y individuals who are highly susceptible to CP and CP, and to work towards developing preventative therapy for these individuals. ..
  57. THE PATHOGENESIS OF AUTOIMMUNITY IN A MURINE MODEL OF PRIMARY BILIARY CIRRHOSIS
    MERRILL E GERSHWIN; Fiscal Year: 2010
    ..abstract_text> ..
  58. Nonalcoholic steatohepatitis and Cytochrome P450 Enzymes
    Naga P Chalasani; Fiscal Year: 2010
    ..Additionally, we examine the relationship between this enzyme activity and the degree of insulin resistance and lipid peroxidation. ..
  59. INTERLEUKIN 6 AND HUMAN BILIARY EPITHELIAL PROLIFERATION
    Anthony Demetris; Fiscal Year: 2006
    ..abstract_text> ..