TITLE

Primary sclerosing cholangitis

AUTHOR(S)
Worthington, Joy; Chapman, Roger
PUB. DATE
January 2006
SOURCE
Orphanet Journal of Rare Diseases;2006, Vol. 1, p41
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9- 1.31/100,000 and point prevalence of 8.5-13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8-30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.
ACCESSION #
30094008

 

Related Articles

  • Rare disease day, conference 29.2.2012 - Presentations.  // Acta Facultatis Pharmaceuticae Universitatis Comenianae;2012 Supplement 7, Vol. 59, p9 

    The article discusses several studies including "National Registry for Cystic Fibrosis (CF) in Slovakia" "Slovak Alliance of Rare Diseases (Slovak RD Alliance), " and "Orphan Drugs: The Approach of European and Slovak Regulatory Institutions." These researches feature several rare diseases, and...

  • Hepatopulmonary syndrome : a rare diagnosis or easily missed entity? Shah, R.; Mousa, O.; John, S. // QJM: An International Journal of Medicine;Jul2014, Vol. 107 Issue 7, p565 

    No abstract available.

  • Rare Disorders and Diseases.  // Exceptional Parent;Aug2008, Vol. 38 Issue 8, p26 

    The article discusses various reports published within the issue, including 22q11 deletion syndrome by Mary Umlauf, and another about isovaleri acidemia by Jana Monaco.

  • Abdominal Ultrasound for Diagnosis of Nonalcoholic Fatty Liver Disease (NAFLD). Mishra, Poonam; Younossi, Zobair M. // American Journal of Gastroenterology;Dec2007, Vol. 102 Issue 12, p2716 

    Nonalcoholic fatty liver disease (NAFLD) is one of the most common causes of chronic liver disease. The progressive subtype of NAFLD or nonalcoholic steatohepatitits (NASH), may progress to cirrhosis and its complications. Unfortunately, accurate noninvasive modalities for diagnosing NASH and...

  • A New Generation Prothrombin Time Method for INR. Horsti, Juha; Uppa, Helena; Vilpo, Juhani A. // Open Medicinal Chemistry Journal;2008, Vol. 2, p11 

    Prothrombin time (PT) is the leading test for monitoring oral anticoagulation therapy (OAT). We sought to determine INR taking into account only active coagulation factors FII, FVII and FX without inhibition in patient plasmas and calibrator kits. We measured PT using a combined thromboplastin...

  • Spider naevi not liver indicator in children.  // GP: General Practitioner;4/28/2006, p10 

    The article reports on a study in Ireland which states that the presence of one or more spider naevi is not a good indicator of liver disease in children. The study of 460 children aged five to 15 years, 34 of whom had chronic liver disease, found that while 80 per cent with cirrhosis had at...

  • Autoantibodies in autoimmune liver disease: biomarkers versus epiphenomena. Selmi, Carlo; Gershwin, M. Eric // Gut;Jun2010, Vol. 59 Issue 6, p6 

    The article examines the role of serum autoantibodies as biomarkers for autoimmune liver disease diagnosis. It was pointed out that amidst the lack of prognostic significance, antimitochondrial antibodies (AMAs) are critical in sensitive and specific diagnosis of primary biliary cirrhosis. A...

  • Successful Application of Extracorporeal Liver Perfusion: A Technology Whose Time Has Come. Fox, Ira J.; Langnas, Alan N.; Fristoe, Lance W.; Shaefer, Mark S.; Vogel, James E.; Antonson, Dean L.; Donovan, Jeremiah P.; Heffron, Thomas G.; Markin, Rodney S.; Sorrell, Michael F.; Shaw Jr., Byers W. // American Journal of Gastroenterology;Nov1993, Vol. 88 Issue 11, p1876 

    We have used extracorporeal liver perfusion (ECLP) to aid in the management of three patients with fulminant hepatic failure (FHF). Organs were used for FCLP only if they would have gone otherwise unused through United Network for Organ Sharing. In all three patients treated, serial serum...

  • Foetal and neonatal alloimmune thrombocytopaenia. Kaplan, Cecile // Orphanet Journal of Rare Diseases;2006, Vol. 1, p39 

    Foetal/neonatal alloimmune thrombocytopaenia (NAIT) results from maternal alloimmunisation against foetal platelet antigens inherited from the father and different from those present in the mother, and usually presents as a severe isolated thrombocytopaenia in otherwise healthy newborns. The...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics