Rapid normalization of severe hypercholesterolemia mediated by lipoprotein X after liver transplantation in a patient with cholestasis - a case report.

Krzysztof Jankowski; Anna Wyzgał; Aldona Wierzbicka; Olga Tronina; Magdalena Durlik; Piotr Pruszczyk

Krzysztof Jankowski Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.;
Anna Wyzgał Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.;
Aldona Wierzbicka Department of Biochemistry and Experimental Medicine, Children's Memorial Health Institute, Warsaw, Poland.;
Olga Tronina Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland.;
Magdalena Durlik Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland.;
Piotr Pruszczyk Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland.;

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.