primary sclerosing cholangitis


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primary sclerosing cholangitis

idiopathic chronic hepatobiliary disease characterized by diffuse inflammation and fibrosis of the extrahepatic biliary system resulting in patchy, irregular stricturing of the bile ducts. Progressive bile duct obliteration to cirrhosis, hepatic failure, and bile duct cancer may occur in up to 50% of patients. Frequently associated with inflammatory bowel disease.

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.

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
References in periodicals archive ?
Diagnosis and management of primary sclerosing cholangitis.
The report reviews pipeline therapeutics for Primary Sclerosing Cholangitis by companies and universities/research institutes based on information derived from company and industry-specific sources
Yearly colonoscopies are often recommended for patients with primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), but the evidence to support early screening has been limited, said Dr.
In 1993, he was diagnosed with a rare liver disease called primary sclerosing cholangitis, the same illness that killed Chicago Bears great Walter Payton.
The recipient was suffering from primary sclerosing cholangitis, a chronic inflammation and fibrosis of all bile ducts.
A Because it is a mouthful to say, primary sclerosing cholangitis might very well become commonly known as "Walter Payton's disease," the same way that amyotrophic lateral sclerosis (ALS) is primarily referred to as "Lou Gehrig's disease.
Payton had suffered from primary sclerosing cholangitis, a rare liver disease that could only be cured by a transplant.
Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrower due to inflammation and scarring.
It also reviews key players involved in the therapeutic development for Primary Sclerosing Cholangitis.
Phase 2 studies with GS-9674 are ongoing in patients with NASH, primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC).
Net proceeds may be used to fund the payment of the cost of the capped call transactions, to fund the ongoing commercialisation of Ocaliva (obeticholic acid, or OCA) in primary biliary cholangitis (PBC), nonalcoholic steatohepatitis (NASH) and primary sclerosing cholangitis (PSC), for general corporate purposes, capital expenditures, working capital as well as prosecution and maintenance of its intellectual property.
It also includes a brief appraisal of IBD in Asian regions and IBD in patients with primary sclerosing cholangitis (PSC), 2 conditions that are not routinely reviewed in pathology literature.

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