Hyperlipoproteinemia type 2
Overview
A rare inherited blood disorder characterized by high levels of betalipoproteins and cholesterol.
Symptoms
* Increased level of beta-lipoproteins * Increased serum cholesterol * Xanthoma
Causes
* High blood fats * Hyperlipidemia * Hereditary hyperlipidemia
Treatment
For type II, dietary management to restore normal lipid levels and decrease the risk of atherosclerosis includes restriction of cholesterol intake to less than 300 mg/day for adults and less than 150 mg/day for children; triglyceride levels must be restricted to less than 100 mg/day for children and adults. The diet should also be high in polyunsaturated fats. In familial hypercholesterolemia, nicotinic acid with a bile acid usually normalizes low-density lipoprotein levels. For severely affected children, portacaval shunt is a last resort to reduce plasma cholesterol levels. The prognosis remains poor regardless of treatment; in homozygotes, myocardial infarction usually causes death before age 30.