Data on the etiology of dilated cardiomyopathy in children (post myocarditis, metabolic, associated with neuromuscular pathology, idiopathic) are presented. Genetic aspects of the disease are covered, a wide spectrum of mutationsin genes encoding proteins of various structures of the cardiomyocyte (sarcomeric complex, cytoskeleton, Z-disks, mitochondria), different variants of inheritance are described. The need for early diagnosis of metabolic cardiomyopathiesis underlined. Criteria for unfavorable prognosis are presented. Modern approaches to medical and surgical treatment of dilated cardiomyopathy are covered.

The bladder is a unique organ that is exposed to various aggressive factors – from mechanical stretching to exposure to toxins, salts and microorganisms. To protect the epithelium of the bladder mucosa, a number of protective mechanisms have evolved in the course of evolution. The violation or absence of these mechanisms can lead to pathological conditions, including the development of urinary tract infection. The study and deep understanding of these mechanisms can contribute to the emergence and improvement of new promising methods of prevention and treatment of urinary tract infections. The presented article details the features of the structure of the urinary bladder mucous membrane and the main mechanisms and factors of its protection against uropathogens.

At present, sensitization to airborne allergens in domestic animals is absolutely rightly considered one of the key risk factors for the development of allergic rhinitis and bronchial asthma, as well as largely determines the development of severe forms of atopic dermatitis and respiratory allergosis. Of particularimportance isthe fact that, unlike othertriggers, the elimination of allergensin domestic animals is often associated with internal resistance from patients. The article presents the current data on the prevalence of sensitization, the properties and clinical significance of the main allergens of cats and dogs, outlines approaches to conducting elimination activities.

The article presents current views on energy metabolism in a living cell, information on the mechanisms of its implementation in norm and in pathology. The methods of diagnostics of intracellular energy disturbances including the assessment of mitochondrial enzymes activity in blood lymphocytes, the level of total and bound carnitine using photoelectrocolorimetry and liquid tandem chromatomass spectrometry methods are discussed in detail. The main clinical manifestations of mitochondrial disorders are described: pathological manifestations on the part of the muscular and central nervous systems, the heart, other somatic organs, the polysystemic nature of lesions. The authors analyze the recent medical publications on the state of energy metabolism in different pathologies in children: with mitochondrial diseases, pathology of the digestive tract, urinary system, vegetovascular dystonia, etc. Data on the ways of correction of energy metabolism disorders using the energy-rich therapy are presented. It is convincingly shown that mitochondrial disorders require L-carnitine preparations in the form of monotherapy or in combination with other drugs.

Objective: to assess the incidence, prevalence and outcomes of end-stage renal disease (ESRD) in children in Belarus, age and gender of patients, etiology of renal diseases leading to ESRD. The study included 121 children, who received RRT as of January 01, 2007 and all subsequent new patients, who started this therapy before December 31, 2016. Results. The incidence of ESRD in  children in Belarus aged 0–14 years for the period 2007–2016 was 7 (6; 8) cases per year (4.8 (4.2; 5.2) per million age related population (pmarp)) and at the age of 0–17 years for the period 2009–2016 – 11 (9; 12) cases per year (5.6 (4.8; 6.8) pmarp). The prevalence of ESRD during the period from 2007 to 2016 increased by 31.7% in  the group of 0–14 years [from 26 patients (18.3 pmarp) to 38 (24.1 pmarp) patients], and by 46.7% aged 0–17 [from 37 (21 pmarp) to 57 (30.8 pmarp)]. The main cause of ESRD was congenital abnormality of kidneys and urinary tract (44.6% of cases). As of December 31, 2016, 75.4% of children were transplanted, 15.8% received peritoneal dialysis and 8.8% – hemodialysis. 5 year RRT mortality rate was 17.9 deaths per 1000 patient-years and main course of death was infection complications (50%). Conclusions. The incidence of ESRD in children in Belarus corresponds to the average of ESPN/ERA–EDTA Register, the prevalence islower, but with a constant growth. The low mortality rate of patients on RRT and high transplantation activity with insufficient number of preemptive transplantations is maintained.

The data on the content and circadian changes in two calcium regulating hormones, parathyroid hormone and calcitonin, as well as on morphological changes in kidney tissue as risk factors for urolithiasis in children with various kinds of concrements in the organs of the urinary system, including bilateral and unilateral nephrolithiasis, concrements of the ureters and the bladder, are presented. Differences were established not only in the quantity, variation in the circadian periodicity of hormones, but also in the ratio of these two hormonesin comparison with the norm. The most pronounced changes were detected with bilateral and unilateral nephrolithiasis.

Objective: evaluation of markers of endothelial dysfunction (endothelin-1 and nitric oxide) and elemental status (zinc, iron, magnesium) in children and adolescents at different stages of chronic kidney disease (CKD). The study includes 104 patients aged 2 to 17 years with different stages of CKD: 63 patients with I stage of CKD, and 26 patients with CKD stage III and 15 patients with CKD V stage. Results: in patients with CKD, endothelin-1 increased with simultaneous decrease in production of nitric oxide in the progression of CKD from I to V stage. The increase of the content of endothelin-1 by 2.5 times determined in patients with stage V CKD, which was accompanied by a decline of the ratio of the concentrations of NO/ET-1 by 5.5 times. In patients with CKD, elemental status impairments were detected, manifested by a decrease in the content of zinc, iron, and magnesium in the serum of blood at the first stage, with the maximum implementation in stage III CKD. Hypertension was detected in 68.3% of patients with CKD. Direct correlations were found between the content of serum iron and magnesium with the level of arterial pressure in patients with stage I CKD. In III and V stage CKD, a direct correlation of serum magnesium with blood pressure level and nitric oxide concentration was established. Conclusion: children with CKD have endothelial dysfunction and elemental status disorders, beginning with the first stage of CKD development, which can be considered as factors determining the progression of the pathological process in the kidneys.

A modern highly informative method for the functional diagnosis of the myocardium is the study of the myocardial strain, i.e. changes in the geometry of the myocardium, evaluated in different directions. In the strain, the deformation (change of the starting, or initial geometry) and the transformation (restoration of the original geometry) are distinguished. The stages of development of systolic myocardial dysfunction (early, deployed and decompensation stages), as well as the processes in the myocardium at each stage were studied. The earliest manifestations of systolic myocardial dysfunction are a decline in the longitudinal strain and compensatory increase in performance of the circular(and radial)strain. The reduction of the global ejection fraction isrevealed only at the stage of decompensation. The standard data on the magnitude of the longitudinal, radial and circular strain in adults are given, as well as own data on the magnitude of the radial and longitudinal strain in children. In 37 children without myocardial diseases at the age from 7 to 17 years, segmental radialstrain on the average was 34±9% (the lowerlimit of the norm is 20%). No significant differencesin the magnitude of radialsegmental strain in groups of children aged 7–11 and 12–17, as well as in the groups of children with body surface area from 0.65 to 1.3 m2 and from 1.31 to 1.73 m2 were revealed. The lowerlimit of the norm for the longitudinalstrain in the study group was –20%.

The aim of the research is to study the state of emotional-volitional function in infants, who were born and living in Lugansk and Donetsk during the military conflict. A dynamic clinical examination was made with the assessment of the rate of emotional-volitional development in 68 children in the area of military conflict. Among them 32 children were breast-fed, forming Ia (the main) group of children, and 36 children were bottle-fed, forming Ib (the comparison group). The second (control) group of our study consisted of 88 children, who were outside the area of military conflict. Among them there were 47 children, who were breast-fed (IIa group), and 41 children, who were bottle-fed (IIb group). The assessment of the level of emotional-volitional development in children was carried out using a standardized clinical-psychological technique called “Gnome”. Results: The level of development of emotional-volitional function in children depends on the type of feeding and the environment. Despite the stressful situation the mother’s milk and physical contact with the mother contributed to higher levels of emotional and volitional development compared to the comparison group of children. But the stress of the war still promoted inhibition of emotional and volitional development, compared with children, who had similar feeding, staying in a peaceful environment.

IXa glycogenosis is the most common of the currently known 15 types of glycogenosis. The disease has X-linked recessive inheritance, is caused by mutations in  the PHKA2 gene localized on the short arm of X chromosome in  the Хр22.13 region. The main manifestations of the disease: stunted growth, increased liver size, episodes of hypoglycemia, fasting ketosis, increased blood levels of cholesterol, low-density lipoproteins, triglycerides, liver transaminases. In many cases, the course of the disease can be relatively mild, which complicates early diagnosis and the timely administration of therapy. Clinical observations of two children from unrelated families with the same homozygous c.884G> A mutation (р.Arg295His, or R295H) in the PHKA2 gene are presented. The similarities and differences in clinical symptoms are emphasized, and the features of patient management are presented.

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Dent’s disease-1 (CLCN5 gene) is a rare X-linked recessive tubulopathy characterized by low molecular weight proteinuria, hypercalciuria, nephrolcalcinosis or nephrolithiasis, proximal tubular dysfunction and renal failure in adulthood. Females are carriers and usually mildly affected. We present an 11-year-old child with nephrocalcinosis and nephrolithiasis with c.2010delG (or p.Asp671fs) mutation in CLCN5 gene which had not previously been reported in the Dent’s disease-1.

Chloride diarrhea is a rare genetically determined disease caused by mutations of the SLC26A3 gene and characterized by the appearance of persistent watery diarrhea from the first days of the child’s life. The disease is accompanied by the development of hypokalemic hypochloraemic alkalosis. Features of the clinical aspects and metabolic disorders in congenital chloride diarrhea determine the need for differential diagnosis with a wide range of pathological conditions, which often leads to late diagnosis and increases the risk of complications. The article presents the clinical analysis of a patient with congenital chloride diarrhea, the criteria for diagnosing the disease, the possible errors in the diagnostic search process, describes the tactics of the child’s management.

The article presents modern ideas about disorders of hydroionic metabolism in children. Many diseases of different etiologies can lead to the development of disturbances in the exchange of water and ions. Depending on the prevailing losses, the main types of dehydration are identified: isotonic, hypotonic and hypertonic. Approaches to the treatment with the help of enteral and parenteral rehydration aimed at correcting not only dehydration but also microelemental disorders are presented. Basic solutions for oral rehydration (ORS-200, Regidron Bio, Humana Electrolyte), as well as special children’s water for use in medical practice and nutrition are presented. The indications for carrying out the infusion therapy are justified.

The article is devoted to the multifarious activities of Genrikh Ivanovich Turner (1858–1941). It is noted that originally G.I. Turner was interested in problems abstracted from orthopedics: acute purulent processes in the right iliac fossa, etc. In 1895, Turner was appointed Professor of  the Department of Desmurgy and Mechanics of  the Military Medical Academy. He revived the teaching of the subject and transmitted it into “broad practical soil”. Genrikh Ivanovich Turner became one of the founders of the Russian orthopedics, the organizer and the head (since 1900) of the first in Russia department and clinic of orthopedics. G.I. Turner gave a lot of power and energy to render assistance to sick children suffering from physical disabilities, restore the working capacity of children with disabilities. For many years he was in charge of the orphanage in St. Petersburg, which was transformed into the G.I. Turner Research Institute of Child Disability in 1932. This institution became an organizational and methodological center for combating child disability in  the Soviet Union. Genrikh Ivanovich was the first in Russia, who raised his voice in favor of  the disabled child, pointed out the need to provide systematic state assistance to disabled children with diseases of the musculoskeletal system.