title: Rapid normalization of severe hypercholesterolemia mediated by lipoprotein X after liver transplantation in a patient with cholestasis - a case report.
abstract: Hypercholesterolemia is a common disorder in adult population, but total cholesterol concentrations beyond 1000 mg/dl occur rarely, and are found in patients with homozygous familial hypercholesterolemia and familial lecithin-cholesterol acyltransferase deficiency, in chronic graft-versus-host disease of the liver, after intravenous infusion of fat emulsion (intralipid), in newborn infants with immature liver function, and in obstructive biliary cholestasis. Cholestasis induces a dramatic increase in plasma cholesterol and the appearance of an abnormal lipoprotein, lipoprotein X (LpX), in the plasma. We report a case of severe hypercholesterolemia mediated by LpX in a patient transplanted for primary biliary cirrhosis (PBC), who was qualified for liver re-transplantation (re-LTx) due to chronic cholestasis. Four months after re-LTx, the cholesterol concentration was normal. The problems in diagnosis and treatment are discussed.
authors: Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.
authors: Krzysztof
authors: K
authors: Jankowski
authors: Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.
authors: Anna
authors: A
authors: Wyzgał
authors: Department of Biochemistry and Experimental Medicine, Children's Memorial Health Institute, Warsaw, Poland.
authors: Aldona
authors: A
authors: Wierzbicka
authors: Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland.
authors: Olga
authors: O
authors: Tronina
authors: Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland.
authors: Magdalena
authors: M
authors: Durlik
authors: Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.
authors: Piotr
authors: P
authors: Pruszczyk
mesh_terms: D002779:Cholestasis / Q000097:blood; D002784:Cholesterol / Q000097:blood; D004655:Emulsions; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications; D006720:Homozygote; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications / Q000378:metabolism*; D007262:Infusions, Intravenous; D008055:Lipids / Q000737:chemistry; D008073:Lipoprotein-X / Q000097:blood*; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000378:metabolism*; D016031:Liver Transplantation / Q000009:adverse effects*; D008875:Middle Aged; D012086:Reoperation; D016896:Treatment Outcome
chemical_list: D004655:Emulsions; D008055:Lipids; D008073:Lipoprotein-X; D002784:Cholesterol