The Lipemia in Familial Plasma Lecithin: Cholesterol Acyltransferase Deficiency

The earlier investigations of a female patient with familial plasma lecithin: cholesterol acyltransferase (IXAT) deficiency showed that a good deal of the triglyceride‐rich lipoproteins had physical properties similar to those of the chylomicrons. Moreover, the heparin‐induced lipoprotein lipase activity (LLA) was distinctly reduced. The present study had the purpose of determining the type of lipemia in LCAT deficiency. For a week the patient had a high fat diet, the next week a high carbohydrate diet. The lipemia was carbohydrate‐induced. The top fraction on the polyvinylpyrrolidone (PVP) density gradient strongly increased when the patient was given a diet extremely rich in carbohydrate. A broad beta‐lipoprotein band followed by a marked trail could be seen by lipid electrophoresis. There were very small amounts of alpha‐lipoprotein during the whole experiment. The results are discussed, particularly with regard to the possible effect of lack of LCAT activity on the plasma lipoproteins.