Семейная гиперхолестеринемия является моногенным аутосомно-доминантным заболеванием, сопровождающимся значительным повышением уровня холестерина в крови и, как следствие, преждевременным развитием и прогрессирующим течением атеросклероза, как правило, в молодом возрасте. Пациенты с семейной гиперхолестеринемией, даже при отсутствии заболеваний, обусловленных атеросклерозом, относятся к группе высокого риска, а при их наличии-к группе очень высокого риска развития сердечно-сосудистых осложнений. Цель-разработка практических рекомендаций для врачей, определение критериев диагностики и современных подходов к лечению семейной гиперхолестеринемии. Материал и ВЕСТНИК СОВРЕМЕННОЙ КЛИНИЧЕСКОЙ МЕДИЦИНЫ 2017 Том 10, вып. 2 73 В ПОМОЩЬ ПРАКТИЧЕСКОМУ ВРАЧУ методы. В рекомендациях рассмотрены этапы оказания медицинской помощи данной группе пациентов в зависимости от возраста и пола. Результаты и их обсуждение. Для каждого этапа приведены возможные виды медицинской помощи. Представлен алгоритм выявления, ведения и лечения больных с семейной гиперхолестеринемией. Заключение. Представленные основные положения методических рекомендаций по организации медицинской помощи больным с семейной гиперхолестеринемией в субъектах Российской Федерации предназначены для врачей общей практики, врачей-педиатров, кардиологов, а также врачей других специальностей. Ключевые слова: семейная гиперхолестеринемия, холестерин липопротеидов низкой плотности, профилактика, атеросклероз, лечение.
The abuse of food of carnivores (meat) by phylogeneticallyI herbivorous Homo sapiens (a patient) initiates atherosclerosis. Addressing biogenetic law of E. Haeckel that ontogeny recapitulates phylogeny (a universal anamnesis), we suggest a diagnostic technique that allows evaluation of the meat diet abuse by a herbivorous Homo sapiens. This technique is based on application of phylogenetic theory of general pathology to clinical practice. The degrees of objective evaluation of nonphysiological overeating of meat are: the first, an increase in the fast plasma content of oleic triglycerides palmitoyl-oleyl-palmitate (POP). The second, hyperglyceridemia + an increase in low density lipoprotein cholesterol (LDL-CL) content. The third, increased plasma content of apoС-III. The fourth, an increase in the concentration of apoВ-48. If electrophoregrams are analyzed and hyperlipoproteinemia (HLP) type is determined according to WHO classification, the first degree of meat overeating is not informative, the second, corresponds to type IV HLP; the third, to type IIb HLP, and the forth, to type V HLP, i.e, the patient diet consists practically of the food of carnivores. Hyperlipoproteinemia coincides with insulin resistance syndrome, hyperglycemia and hyperinsulinemia, which is based on blood increase of fatty acids in the form of polar unesterified fatty acids (UFA). According to phylogenetic theory of general pathology, in vivo cells do not internalize glucose if there is a possibility to internalize UFA. Preventive examination allows evaluation of disorders in the biological function of trophology (food consumption). Thus, the use of different methods in the analysis of this function offers evaluation of the effectiveness of diet therapy from the level of disorders when treatment was started.
Phylogenetically late arterial intima of the elastic type contains no proteins for the transfer of ligandless oxidized low density lipoproteins (LDLP) for sedentary macrophages adsorbed on the matrix. Phylogenetically early cells realize the extracellular digestive reaction by releasing proteolytic enzymes (metalloproteinases) into intimal matrix that hydrolize matrix proteoglycans, adsorbed ligandless LDLP, detritus, and complete lysosomal hydrolysis of the most hydrophobic polyenic cholesterol esters (poly-ECS). Smooth muscle cells migrate from the middle muscular layer of the arterial wall, change their contractile phenotype to secretory one, and synthesize in situ de novomatrix proteoglycans. The arterial wall has three layers (monolayer endothelium, intimal media (smooth muscle cells), and adventitia) only in elastic type arteries. It is desirable to elucidate functional differences between phylogenetically early sedentarymacrophages and monocytes-macrophages of later origin and understand whether theydepends on specific features of activity of scavenger eceptors, CD36 translocases, expression of acid hydrolases synthesis for poly-ECS or realization of the extracellular digestion reaction. We believe that formation of atheromatous masses takes place in the matrix of arterial intima rather than in lysosomes taking into account limited possibilities for monocytes-macrophages to realize endocytosis of ligandless LDLP from the matrix. Given that atheromatosis is a syndrome of deficit of essential polyenic fatty acids (PFA) in the cells, intimal atheromatosisshould be regarded only as partial utilization of excess PFA in the matrix of elastic type arteries. At later stages of phylogenesis, intima was formed from media smooth muscle cells.
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