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Information

This rare autosomal recessive disorder is due to the absence of apo CII, an essential cofactor for lipoprotein lipase. As a result, chylomicrons and triglycerides accumulate and cause manifestations similar to those in lipoprotein lipase deficiency.

Treatment: Isolated Hypertriglyceridemia

Pts with severe hypertriglyceridemia should be placed on a fat-free diet with fat-soluble vitamin supplementation. Pts with moderate hypertriglyceridemia should restrict fat, carbohydrate, and alcohol intake. In those with familial hypertriglyceridemia, fibric acid derivatives, omega-3 fatty acids, or niacin can be administered if dietary measures fail (Table 178-2).

Outline

Section 13. Endocrinology and Metabolism