Hyperlipoproteinemia Type I

What is Type I Hyperlipoproteinemia?

Type I hyperlipoproteinemia is an abnormally elevated level of lipids and / or lipoproteins in human blood. Type I hyperlipoproteinemia is very rare.

Causes of Type I Hyperlipoproteinemia

Type I hyperchilomycronemia, or fat-induced lipemia; due to the lack of the enzyme lipoprotein lipase, usually inherited in an autosomal recessive manner.

Pathogenesis during Type I Hyperlipoproteinemia

It is characterized by a high plasma content of chylomicrons and triglycerides. cholesterol levels may be normal or slightly elevated (hypercholesterolemia). Coefficient of cholesterol: triglycerides less than 0.2. When the patient’s blood is standing in the refrigerator after 1224 hours, a cream-like layer consisting of chylomicrons is formed over the transparent plasma.

Symptoms of Type I Hyperlipoproteinemia

Hyperlipidemia increases dramatically after taking fat and decreases after its strict limitation. History (sometimes since childhood) – abdominal pain, recurrent pancreatitis.

Clinical manifestations occur at the age of 10 years; characteristic deposits of lipids in the skin in the form of eruptive xanthomas, as well as in the liver and spleen, which is manifested by hepatosplenomegaly. Often there are abdominal pain, pancreatitis. The development of atherosclerosis is not typical.

Diagnosis of Type I Hyperlipoproteinemia

To recognize the existing type of lipoproteinemia, it is usually sufficient to simply determine the level of lipids in the plasma in combination with the clinical examination data.

Treatment of Type I 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.