primary biliary cirrhosis

(redirected from Cholestatic liver disease)
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primary biliary cirrhosis

[¦Prīm·ə·rē ‚bil·ē·er·ē sə′rō·səs]
(medicine)
A slowly progressive disease primarily of middle-aged women, caused by an autoimmune destruction of bile ducts that begins as inflammation in and around larger intrahepatic bile ducts and eventually results in liver cell damage.
References in periodicals archive ?
Cholestasis is the pivotal hallmark of the so-called chronic cholestatic liver diseases (CCLDs), but it may also occur in the advanced stage of other chronic liver diseases (CLDs), such as alcoholic liver disease, nonalcoholic fatty liver disease, and chronic hepatitis B and chronic hepatitis C [2].
The diagnosis of PBC was made according to the diagnostic criteria for PBC in the American Association for the Study of Liver Diseases Practice Guidelines: [sup][10],[13] (1) Presence of cholestatic liver disease including jaundice, fatigue, and pruritus with abnormally high levels of cholestatic parameters such as serum alkaline phosphatase (ALP), serum bilirubin, and gamma-glutamyl transpeptidase (GGT); (2) absence of biliary obstruction as assessed by ultrasound or endoscopic retrograde cholangiopancreatography; (3) serum positive AMA titer 1:1000 and type M2 AMA positive; (4) absence of serological markers for hepatitis B and C virus and HIV infection by immunoassays.
Vitamin E deficiency is common in cholestatic liver disease.
Primary sclerosing cholangitis is the second most common cause of chronic cholestatic liver disease in adults.
The R&D Leadership Team will highlight a number of programs in Shire s research portfolio, as well as provide updates on several clinical programs in development, including SHP606 (lifitegrast) in Dry Eye Disease, SHP625 (LUM001) in Cholestatic Liver Disease, SHP626 (LUM002) for adults with Nonalcoholic Steatohepatitis (NASH), and SHP607 for the prevention of Retinopathy of Prematurity (ROP).
Lumena said that INDIGO an open-label, Phase 2 clinical trial, will enroll approximately 12 patients under the age of 18 who have been diagnosed with PFIC, and is designed to evaluate the efficacy and safety of LUM001 for the treatment of cholestatic liver disease in patients with PFIC.
Our patient had all of these features in the presence of chronic cholestatic liver disease.
LAP was found to be elevated in papillary adenocarcinoma of bile duct (33) and in cholestatic liver disease (34).
Risk factors include breastfeeding and the presence of malabsorptive diseases such as cystic fibrosis and cholestatic liver disease, which prevent adequate consumption of dietary vitamin K.
ALP enzyme is found mainly in the bile ducts of liver and increase in its level can indicate an obstructive or cholestatic liver disease.
AST, ALT and alkaline phosphatase tests are most useful to make the distinction between hepatocellular and cholestatic liver disease.