Hyperlipoproteinemia, Type Iv

Description

On a regular diet patients with type IV hyperlipoproteinemia demonstrate increased plasma VLDL. Plasma triglycerides are persistently increased, while plasma cholesterol and phospholipids are usually within normal limits. Precocious atherosclerosis, abnormal glucose tolerance, and atheroeruptive xanthoma may occur. The disorder is undoubtedly heterogeneous and the phenotype strongly influenced by environmental factors, particularly carbohydrate and ethanol consumption.

Clinical Features

In an old American family living in New England, Schreibman et al. (1969) described hyperprebetalipoproteinemia behaving as an autosomal dominant with reduced penetrance. Although triglyceride levels as high as 2000 mg per 100 ml were observed in some children of this family, precocious atherosclerosis was not observed. The absence of obesity and glucose intolerance may account for the favorable prognosis. Goldman et al. (1972) emphasized the association of rheumatic manifestations.