| Dictionary, Encyclopedia and Thesaurus - The Free Dictionary 3,918,323,704 visitors served. |
Dictionary/ thesaurus | Medical dictionary | Legal dictionary | Financial dictionary | Acronyms | Idioms | Encyclopedia | Wikipedia encyclopedia | ? |
primary sclerosing cholangitis |
Also found in: Acronyms, Wikipedia | 0.01 sec. |
|
|
Primary sclerosing cholangitis
A chronic disease in which it is believed that the immune system fails to recognize the cells that compose the bile ducts as part of the same body, and attempts to destroy them. It is not clear what exactly causes the disease, but it is frequently associated with another inflammatory disease of the digestive tract, ulcerative colitis. The inflammation of the ducts eventually produces formation of scar tissue, causing multiple areas of narrowing (strictures) that block bile flow and lead to bacterial infection. Liver transplant gives the best chance for long-term survival. Mentioned in: Cholangitis
primary sclerosing cholangitis, a progressive chronic fibrosing inflammation of the bile ducts of unknown cause, occurring most commonly in young men and frequently in association with chronic ulcerative colitis. It also occurs as a complication of HIV infection. See also AIDS cholangiopathy. primary sclerosing cholangitis Sclerosing cholangitis A chronic idiopathic progressive liver disease characterized by inflammation, necrosis, fibrosis and obliteration of intrahepatic and extrahepatic bile ducts, resulting in cholestasis,
cirrhosis, portal HTN, liver failure, often associated with other autoimmune diseases–eg, Crohn's disease, ulcerative colitis, Addison's disease Epidemiology PSC is uncommon–1 to 6/105, US; it is the 4th most
common indication for liver transplant; 75% of Pts have IBD; 70% of Pts are ♂, average age 39 Clinical Often asymptomatic until end-stage liver disease; as albumin ↓ and BR ↑, pruritus, fatigue, jaundice, and weight loss dominate
clinical picture, ± accompanied by fever, chills, night sweats, right upper quadrant pain Imaging Multiple zones of narrowing and dilatation– “beading” of bile duct by ERCP and transhepatic cholangiography Lab ↑ Alk
phos, ↑ aminotransferase, hypergammaglobulinemia, ↑ IgM Management Azacytidine may ↓ need for transfusion, iron chelation–early use of deferiprone, corticosteroids, penicillamine, MTX; ursodiol is useless Prognosis Median
survival after diagnosis, 12 yrs. See Primary biliary cirrhosis. Want to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit the webmaster's page for free fun content. |
|
| Medical Dictionary |
| Free Tools: |
For surfers:
Free toolbar & extensions |
Word of the Day |
Help
For webmasters: Free content | Linking | Lookup box | Double-click lookup |
|---|