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). |
Section 13. Endocrinology and Metabolism