progressive familial intrahepatic cholestasis


ABCB11

A gene on chromosome 2q24 that encodes an ATP-binding cassette (ABC) transporter superfamily of integral membrane proteins, MDR/TAP subfamily, which is involved in multi-drug resistance and is major canalicular bile salt export pump
Molecular pathology ABCB11 mutations cause a type of progressive
familial intrahepatic cholestasis of early (infancy) onset.

cholestasis

, cholestasia (ko?le-sta'sis) (ko?le-sta'zh(e-)a) [ chole- + stasis] Arrest of the flow of bile. This may be due to intrahepatic causes, obstruction of the bile duct by gallstones, or any process that blocks the bile duct (e.g., cancer). cholestatic (ko?le-stat'ik), adjective

intrahepatic cholestasis of pregnancy

Abbreviation: ICP
A complication of approx. 1% of pregnancies in which elevated levels of pregnancy hormones cause obstruction to bile flow within the liver. Levels of bilirubin rise in the maternal circulation during the second and third trimesters of pregnancy, causing itching, bile staining of urine, and a lighter stool color than normal. ICP increases the risk of preterm delivery of the fetus and of bleeding disorders and loss of life of the fetus. Synonym: obstetric cholestasis

obstetric cholestasis

Intrahepatic cholestasis of pregnancy.

progressive familial intrahepatic cholestasis

Byler disease.