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Restrictive cardiomyopathy often results in chronic heart failure. Knowledge of family history and genetic examination are extremely important for the early diagnostics of a family restrictive cardiomyopathy and prescription of non-specific therapy. The article describes clinical case of familial restrictive cardiomyopathy with a burdened family history.

Malnutrition is a common problem in children with congenital heart defects, regardless of the type of defect and the presence / absence of cyanosis. Children born with heart defects form a high-risk group of nutritional deficiency. Surgical treatment of congenital heart defects is often accompanied by metabolic changes and increased needs of energy and protein. Correction of nutritional status in children with congenital heart diseases improves the condition of patients after cardiac surgery. The purpose of our study was to assess the nutritional status of children with congenital heart defects hospitalized to the cardiac surgery department, and to correct their enteral nutrition. The authors analyzed anthropometric data on the Z-score (weight – age) of children admitted to the department for examination and correction of heart disease. The authors concluded that there is need for further studies to assess the impact of changes in nutritional status on the incidence and mortality of patients with congenital heart defects and the possibility of optimal correction of nutritional status.

The article describes medical, scientific, pedagogical activity of the outstanding Kazan scientist, Honored Scientist of the RSFSR, Yefim Moiseevich Lepsky. Ye.M. Lepsky made a great contribution both to the development of the Kazan pediatric school and to the development of Russian pediatrics in general.

Aim. To study the specifics of lead, zinc, copper, magnesium and calcium metabolism in children and adolescents with essential arterial hypertension living in different ecological settings. Methods. 100 children and adolescents aged 13-17 years with arterial hypertension, living in «industrial» (52 patients) and «residential» (48 patients) areas were examined in cardiology ward. 33 healthy children and adolescents comparable by age and gender were included as healthy controls. According to 24-hour blood pressure monitoring, groups with stable, labile arterial hypertension and «white coat hypertension» were formed. The levels of elements in 24-hour urine and in serum were measured by atomic absorption spectrophotometry. Results. In children and adolescents with stable arterial hypertension living in industrial areas, parameters of 24-hour blood pressure monitoring (mean 24-hour and mean daytime systolic blood pressure values, time of systolic hypertension, mean 24-hour and mean daytime blood pressure values) were higher compared to the same parameters in children from residential areas, the difference was statistically significant. The study of elements metabolism revealed increased lead exposure in children with essential arterial hypertension living in industrial areas, and misbalance of essential elements in those patients compared to the same parameters in children from residential areas. Conclusion. Results suggest increased exposure to arterial hypertension and marked misbalance of essential elements in children and adolescents with essential arterial hypertension living in industrial areas.

Aim. To study the features of element homeostasis in children and adolescents with different variants of essential arterial hypertension. Methods. The clinical and functional variant of arterial hypertension was established based on the data of 24-hour monitoring of the arterial blood pressure. Investigation of the element content in the blood serum and daily urine was performed using the method of atomic absorption spectrophotometry. The clearance and the excreted fraction of the investigated elements were measured. Results. Revealed was an increase of the content of lead in the blood serum, an increase in lead excretion with urine, an increase in its clearance and excreted fraction in patients with stable arterial hypertension. It has been shown that for children and adolescents with a variety of clinical and functional variants of arterial hypertension characteristic is an excess of the serum content of a toxic element (lead) over the content of an essential element (zinc), and these changes are most pronounced in the group with stable arterial hypertension. In children and adolescents with the increase in arterial blood pressure registered was a decrease in the content of zinc and magnesium in the blood serum, whereas the disturbances of element homeostasis relate to the metabolic type. The increase in the content of copper and calcium in blood serum of patients with high blood pressure is also a manifestation of the metabolic type of element homeostasis disturbances. Conclusion. The increased level of lead in blood serum in combination with its enhanced excretion may be a cofactor in the development of essential arterial hypertension in childhood and adolescence.