Abstract
Intrahepatic cholestasis and secondary hyperlipidemia developed in a forty-one years old woman. Laboratory data, histology of the bioptic liver specimen and endoscopic retro grade cholangiography were consistent with intrahepatic cholestasis. Jaundice continued for 17 months inspite of steroid therapy. During this treatment, each level of serum lipid fractions increased remarkably, such as cholesterol 3240mg/dl, triglyceride 608mg/dl, phospholipid 206mg/dl, accompaning elevated serum alk. phosphatase (903u), γ-glutamyl transpeptidase (4548u), leucine aminopeptidase (258u) and serum bile acid proportionately. Furthermore, electropholetic pattern of serum lipoprotein showed an abnormal running band of low-density lipoprotein (LDL) which seemed to be influenced by increased lipoprotein-X (Lp-X). Sequential addition of cholestyramin and diet therapy resulted in the improvement of hyperlipidemia.
Although her clinical course and laboratory data indicated to be chronic cholestatic liver disease, this case should be diagnosed of variant acute intrahepatic cholestasis from the histology.
