Hyperlipoproteinemia Type II

What is Type II Hyperlipoproteinemia?

Hyperlipoproteinemia type II (hypercholesterolemia) accounts for about 30% of cases of hyperlipidemia, associated with a decrease in catabolism or increased synthesis of beta-lipoproteins.

Causes of Type II Hyperlipoproteinemia

Type IIa hyperbetalipoproteinemia is inherited in an autosomal dominant manner.

Pathogenesis during Type II Hyperlipoproteinemia

Hereditary hyperlipoproteinemia type IIa develops as a result of mutation of the LDL receptor gene (0.2% of the population) or the apoB gene (0.2% of the population).

Symptoms of Type II Hyperlipoproteinemia

Clinical manifestations in homozygotes occur in childhood, in heterozygotes – in the age of over 30 years. Xanthomas in the region of the Achilles tendon, extensor tendons of the feet and hands, periorbital xanthelasmas are characteristic. Signs of early atherosclerosis are often noted, deaths from myocardial infarction in children and adolescents are described.

Sometimes combined with the lipid arc of the cornea and xanthomatosis. It is characterized by a high risk of rapid and early (even at the 2-3rd decade of life) development of atherosclerosis or sudden death.

Diagnosis of Type II Hyperlipoproteinemia

In the blood, the content of beta-lipoproteins is increased, the amount of cholesterol is sharply increased, the concentration of triglycerides is normal, the coefficient is cholesterol: triglycerides is more than 1.5. The blood plasma after standing in the refrigerator for 12-24 h remains transparent.

Treatment of Type II Hyperlipoproteinemia

Treatment is reduced to the pathogenetic correction of metabolic and clinical syndromes.

For patients with primary and secondary hyperlipoproteinemia and normal body weight, 4-fold food intake is recommended, for obesity 5-6-fold, because rare meals contribute to an increase in body weight, a decrease in glucose tolerance, the occurrence of hypercholesterolemia and hypertriglyceridemia. The main caloric intake should be in the first half of the day. for example, with 5 meals a day, the 1st breakfast should be 25% of the daily calories, the 2nd breakfast, lunch, afternoon snack and dinner, respectively, 15, 35, 10 and 15%. General strengthening therapy is also carried out, with obesity sufficient physical activity is necessary.

In type I hyperlipoproteinemia, heparin and other hypolipidemic agents have no effect. In pediatric practice, it is preferable to use drugs of a milder action: cholestyramine, clofibrate, etc. In some cases, anorectic drugs are prescribed for easier adaptation of the patient to the diet for a short time.

Effective treatments for alipoproteinemia and hypolipoproteinemia have not been developed.