The article shows the effectiveness of the complex treatment (angiotensin converting enzymes inhibitors, blockers, diuretics and cardiac glycosides) in children with dilated cardiomyopathy with an early debut. The dynamic observation of changes in morphological and functional cardiac parameters was carried out and we studied the content of matrix metalloproteinases in 41 patients on the background of the therapy. The statistically significant positive effect of the combined therapy on the studied parameters after 1–1,5 years of the continuous treatment has been demonstrated.Key words: dilated cardiomyopathy, matrix metalloproteinases, treatment, children.

The article shows the effectiveness of the complex treatment (angiotensin converting enzymes inhibitors, blockers, diuretics and cardiac glycosides) in children with dilated cardiomyopathy with an early debut. The dynamic observation of changes in morphological and functional cardiac parameters was carried out and we studied the content of matrix metalloproteinases in 41 patients on the background of the therapy. The statistically significant positive effect of the combined therapy on the studied parameters after 1–1,5 years of the continuous treatment has been demonstrated. Key words: dilated cardiomyopathy, matrix metalloproteinases, treatment, children.

The review highlighted problems of reorganization of myocardical contractile and cytoskeletal proteins in cardiomyopathy (CM). The role of the genetic factors coding contractile proteins, proteins of thin and thick filaments, and also extracellular matrix proteins in processes of formation and development of hypertrophic (HCM) and dilated (DCM) cardiomyopathy are analyzed. The mechanisms responsible for the changes in cardiac proteins on regulation involved into force generation, its transfer, recycling ATP, impairments in transmembranal signals, that finally lead to cardiac cell dysfunction determining various manifestations of CM are considered.

The article is devoted to an important section of the pediatric cardiology — dilated cardiomyopathy (DCMP). This is largely due to the spread of the given disease, its possible progress and tendency to the formation of the chronic heart failure (CHF). The progress in the research of the DCMP'accompanied CHF formation and development processes is the studies of the extracellular matrix status (EM), which is defined by the activity of the matrix metalloproteinases (MMP) and their tissue inhibitors (TIMMP). This article describes the complex clinical and biochemical studies of the serum MMP'1, MMP'2, MMP'9 and TIMMP'1 levels in children with DCMP at different stages of the illness course. MMP and their inhibitor concentration were defined by means of the enzyme'linked immunosorbent assay (ELISA). Based on the results from the morphofunctional studies and data of the biochemical analyses, the authors determined the importance of changes in the isomeric EM composition pertaining to the disturbances of the systolic and diastolic functions, as well as in heart remodeling for such children . Key words: chronic heart failure, dilated cardiomyopathy, extracellular matrix, matrix metalloproteinases, tissue inhibitors of the matrix metalloproteinases.

The article is devoted to an important section of the pediatric cardiology — dilated cardiomyopathy (DCMP). This is largely due to the spread of the given disease, its possible progress and tendency to the formation of the chronic heart failure (CHF). The progress in the research of the DCMP'accompanied CHF formation and development processes is the studies of the extracellular matrix status (EM), which is defined by the activity of the matrix metalloproteinases (MMP) and their tissue inhibitors (TIMMP). This article describes the complex clinical and biochemical studies of the serum MMP'1, MMP'2, MMP'9 and TIMMP'1 levels in children with DCMP at different stages of the illness course. MMP and their inhibitor concentration were defined by means of the enzyme'linked immunosorbent assay (ELISA). Based on the results from the morphofunctional studies and data of the biochemical analyses, the authors determined the importance of changes in the isomeric EM composition pertaining to the disturbances of the systolic and diastolic functions, as well as in heart remodeling for such children.Key words: chronic heart failure, dilated cardiomyopathy, extracellular matrix, matrix metalloproteinases, tissue inhibitors of the matrix metalloproteinases.

Restrictive cardiomyopathy (RCMP) is heart disorder with unclear etiology; it can be characterized as disease with disorder of diastolic myocardium function of left ventricle, conditioned by restriction. The chronic heart failure as a syndrome of RCMP can develop as a result of disbalance in system of complex biochemical, structural, and geometrical mechanisms of myocardium re-modeling. Extra cellular matrix play significant role in heart structure and geometry breaking. The destruction of heart is realized by matrix metalloproteinases (MMP). The activity of MMP, in its turn, is controlled by its tissue inhibitors. The present study analyzed the role of MMP in process of collagen’s synthesis and catabolism deregulation, myocardium fibrosis, change of heart chambers, and development of diastolic dysfunction in children with RCMP.Key words: children, chronic heart failure, restrictive cardiomyopathy, matrix metalloproteinases.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(5):36-39)

The review summarizes information about changes of extracellular matrix (ECM) in cardiovascular diseases. Special attention is paid to different groups of extra cellular matrix proteins (collagen I and III type, fibronectine) in the development of cardiac fibrosis in chronic heart failure. The role of matrix metalloproteinases in degradation of components of ECM is analyzed. Interrelationship between matrix metalloproteinases and their tissue inhibitors in fibrosis and cardiac structural chances is analyzed.

В статье отражены современные взгляды на процессы фиброзирования легочной ткани при формировании бронхолегочной дисплазии с участием матриксных металлопротеиназ и их ингибитора у недоношенных детей, получивших и не получивших терапию сурфактантом в раннем неонатальном периоде.

Complex clinical-laboratory investigation of children with congestive heart failure (CHF) developed on the basis of dilated cardiomyopathy and hypertrophic cardiomyopathy has been carried out. The development of CHF in children with cardiomyopathy was accompanied by changes of blood serum lactate, MB isoform of creatine phosphokinase, TNF-6, interleukin-6 (IL-6) (but not IL-2), and soluble receptors for IL-2 and IL-6.

The content of ionised calcium (Ca2+), calmodulin and total maintenance of hydrolyzates of phosphatidylinositol (inositolphosphate (IP) and diacylglycerol (DG)) in erythrocytes and in myocardium were investigated in children with different forms of ectopic arrhythmias (EA). Disturbances in maintenance of indicated biologically active substances were revealed in patients with EA as compared with healthy children. It was determined that regulation of cellular homeostasis was depended from clinical course of EA. Our results indicate that IP and DG is of great importance in generation and conduction of electric impulses in myocardium by regulation of cellular calcium metabolism in children with EA.

Regulation of intracellular calcium homeostasis by means of secondary mediators calmodulin and 4,5-inositol phosphates (products of phosphatidyl inositol hydrolysis) was studied in children with ectopic forms of arrhythmias. Alterations in the system calmodulin-Ca2+ and the activity of phosphoinosite metabolism correlated highly with clinical manifestations of the arrhythmias. The data obtained suggest that a number of molecular mechanisms and responsible for arrhythmias were realized in cell membranes and depended on a complex of intracellular messengers affected within various steps of the hormonal signal transmission.

The state of membranes of erythrocytes was studied in children with various ectopic arrhythmias before and after physiotherapeutic procedures. Content of total phospholipid, its composition, and cholesterol level in membranes of erythrocytes were measured. Concentrations of lonized calcium, calmodulin and total content of inositolphosphates in erythrocytes and In myocardial tissue were also determined. Before physiotherapy decrease of phospholipid/cholesterol coefficient and dysbalance in contents of separate phospholipid fractions were accompanied by rise In cellular functional activity evidenced by increases of Intracellular levels of ionized calcium, calmodulin and of total Inositolphosphate content. Electromagnetic therapy promoted improvement of clinical condition and sinus rhythm restoration in 86% of patients. In most cases this was accompanied by stabilization of cellular membranes and normalization of mechanisms of regulation of cellular metabolism and of calcium homeostasis in particular. Possible mechanisms of action of physiotherapeutic methods on molecular organization of cellular membranes and on processes of regulation of intracellular metabolism are discussed.

Importance of calmodulin and Ca2+ in membranes of erythrocytes and myocardium was studied in children with ectopic forms of cardiac arrhythmia--extrasystole, paroxysmal and nonparoxysmal tachycardia. Activation of lipid peroxidation and shifts in cytomembrane phospholipid spectrum were shown to be involved in impairments of calcium homeostasis regulation, which is responsible for control of electromechanical parameters and may be related to deterioration of myocardial electrophysiological properties.

The resistance of the rat's organism to emotional stress is determined by alterations of the heart's electric stability. The condition of lipid oxidation is accompanied by alterations of cholesterol and ionised calcium concentrations. Such alterations were expressed in Fisher rats to a greater extent. It conformed to the morphophysiological peculiarities of this group of animals and confirmed its considerable species sensibility.