[Primary hypercholesterolemia: mechanisms of its development in man].

The review is devoted to hypercholesterolemia which is one of the leading risk factors for IHD. By its origin it can be primary and secondary. Three major mechanisms of primary hypercholesterolemia in humans are discussed. They are: low activity of LDL receptors, reduced affinity of LAL for receptors and overproduction of lipoproteins containing apo B. Depending on mechanism nonidentical molecular defects leading to cholesterol imbalance in cells or in circulating lipoproteins are occurred. The understanding of the nature and mechanisms of hypercholesterolemia development is of great clinical value, because having determined molecular defect and using drugs combinations the majority of patients are a success to have their lipids normal. Only in the case of homozygous familial hypercholesterolemia medicamentous treatment becomes secondary and the principal therapeutic methods are plasmapheresis or selective LDL apheresis. Gene therapy as a method of homozygous familial hypercholesterolemia correction is in forthcoming future.