Familial Lecithin: Cholesterol Acyltransferase Deficiency: Studies on Lipid Composition and Morphology of Tissues

Abstract

AbstractKidney transplantation was performed on a patient with familial LCAT deficiency. She died 5 months later from various infections. Lipid deposition was demonstrated in spleen, liver, and kidney, accumulation of unesterified cholesterol being most marked. The lipid composition of an atheroma indicated that lipoproteins relatively rich in free cholesterol and in saturated and monounsaturated fatty acids had been filtered from the plasma to the atheromatous lesion. The half-life of the plasma esterifying activity in this patient was 4.6 days.Key Words: Atheromaatherosclerosischolesterolinborn error of lipid metabolismkidney transplantationlecithincholesterol acyltransferase (LCAT)lipid depositionphospholipidsrenal failure