In the preceding developmental period, information technologies in pediatrics found rather wide application in various fields (prophylactic examinations, diagnosis, electronic medical records, and specialized registers). At present, there are clearly prospects that are associated with transition to e-health and person-centered data integration. Electronic health records in their modular construction will ensure the formation of a variety of problem-oriented registers based on primary information entered once. Portable electronic devices intended for home use, by transferring the data to processing centers and physicians, will ensure constant monitoring of the health of certain contingents of children and responsiveness of critical changes of monitored physiological parameters. Built-in EHR assisted decision support system will serve as a guide for physicians in the diagnosis and treatment of children, which is particularly important to choose medicines.

The paper summarizes research material on viral or bacterial infections and inflammatory diseases in infants, which are represented by the specific features of the development of the immune system as a whole, including the interferon system. This age period is characterized by physiological humoral immune deficiency; decreased activation of the complement system; insufficient synthesis of cytokines, including interferons; T-cell immunosuppression; impaired intercellular cooperation, and a weak functional activity | of natural killer cells and phagocytes. In this period, information on environmental antigens is accumulated in the presence of a primary immune response, which does not ensure a steady anti-infectious defense. In spite of the available large arsenal of antiviral agents and methods to prevent viral and bacterial infections and inflammatory diseases, there is a search for novel approaches to treating infectious diseases with consideration for the specific features of the immune status in infants.

The paper considers the problem of diagnosing the heterogeneous forms of mitochondrial cardiomyopathies associated with impaired oxidative phosphorylation and decreased activity of the mitochondrial electron transport system due to mutations in mitochondrial or nuclear DNA genes. It is emphasized that mitochondrial cardiomyopathies rarely present as an isolated myocardial lesion and more frequently serve as a manifestation of multisystem disease. The paper provides the characteristics of cardiomyopathies in some mitochondrial syndromes and shows the possibilities of treating this disease.

The review of literature analyzes the results of the current scientific works highlighting the role of prolactin in the body’s metabolic processes and its effect on the cardiovascular system.

Objective: to reveal the adaptive features of the cardiovascular system in newborn infants with intrauterine growth retardation (IUGR) on the basis of a clinical instrumental study.

Subjects and methods. A study group included 100 newborn infants with IUGR; a control group consisted of 40 babies with normal anthropometric measurements at birth. Medical history and clinical data and electrocardiographic and echocardiographic findings were analyzed.

Results. All the examinees with IUGR had manifestations of cardiovascular system dysadaptation. There was a high rate of electrocardiographic changes, such as cardiac arrhythmias; low voltage; systolic overload of the right heart and left ventricle; signs of ventricular hypertrophy; and transient myocardial ischemia. The specific features of cardiac hemodynamics were decreased sizes of the left ventricle, lower parameters of its systolic function, and longer functioning of fetal communications.

Conclusion. IUGR is associated with the development of cardiovascular system dysadaptation syndrome, which is due to prior perinatal hypoxia. The findings necessitate a follow-up of children by involving a cardiologist.

Objective: to reveal the specific features of the lymphocyte enzyme status in newborn infants with hypotrophic type of intrauterine growth retardation under the conditions of Yakutia.

Subjects and methods. A total of 137 full-term newborns (including 28 babies with intrauterine growth retardation) whose parents were Yakutsk newcomers were examined on 1, 3 and 5 days of life. The cytochemical activity of lymphocyte enzymes, by determining the level of and time course of changes in succinate dehydrogenase (SDH), glycerol-3-phosphate dehydrogenase (GPDH), lactate dehydrogenase (LDH) and its aerobic isoenzyme (LDH-H), NADFH2-diaphorase, monoamine oxidase (MAO), and acid phosphatase (AP), were analyzed.

Results. It was ascertained that the activity of SDH, GPDH, and LDH was higher on 5 day of life and that of NADFH2-diaphorase was lower in infants with intrauterine growth retardation than in apparently healthy neonates. Desynchronosis of the metabolic pathways was established in the dynamics of early ontogenesis.

Conclusion. It is necessary to rationally correct standard treatment regimens, by taking into account the found metabolic features in infants with intrauterine growth retardation under the conditions of Yakutia.

The common cause of mitochondrial diseases is mutations in the POLG1 gene encoding the catalytic subunit of DNA-polymerase gamma that is responsible for the replication and repair of mitochondrial DNA. Diseases associated with POLG1 gene defects are characterized by obvious clinical polymorphism and may resemble some mitochondrial syndromes and other hereditary and nonhereditary diseases, which gives rise to diagnostic difficulties. Another characteristic feature is that phenotypic manifestations are of diversity in various patients having the same mutation in the POLG1 gene. The paper describes mitochondrial encephalomyopathy, caused by homozygous mutation in the polymerase gamma (POLG1, p.L304R), with different clinical manifestations and a poor outcome in patients from two different families.

A total of 141 patients aged 10 to 17 years were examined. Doppler ultrasonography was carried out to assess blood flow in the intracranial (basilar and middle cerebral arteries) and extracranial (common and internal carotid arteries, vertebral arteries) cerebral arteries and cerebral vein (tentorial venous sinus) and the velocity characteristics of the flow and the level of peripheral vascular resistance were analyzed in all the children. Normal weight children with stable hypertension were found to have high peripheral vascular resistance in the beds of intracranial (basilar and right middle cerebral arteries) and extracranial arteries with the phenomena of hyperperfusion in the bed of the common carotid artery. Obese children having different stages of hypertension were, on the contrary, observed to have lowered peripheral resistance in the intracranial arteries and increased peak systolic blood flow velocity in the tentorial venous sinus. The found cerebral hemodynamic differences may suggest that obese children have secondary hypertension.

The ultrasound and angiographic parameters of the right chambers of the heart were analyzed in children with pulmonary arterial hypertension (PAH) associated with congenital heart defects. The investigation was conducted in relation to the functional class (degree) of PAH. Right cardiac remodeling was found to depend to a greater extent on afterload (pulmonary artery systolic pressure and pulmonary vascular resistance); moreover, diastolic dysfunction developed just in moderate (Functional Class II) PAH. It was ascertained that there was a prompter development of PAH with myocardial systolic and diastolic dysfunction in the presence of congenital malformations with arterial hypoxemia.

The paper describes a clinical case of a family, in which its 5 members (4 women and 1 man) were found to have the new missence mutation c. 3098G>A, p.(1033D) in the gene encoding the α5-chain of type IV collagen (COL4A5) responsible for the development of X-linked Alport syndrome. At the same time, all the four female carriers were observed to have had kidney injury since infancy (at the age of 3 years), an age progressive glomerular filtration rate reduction to 78 ml/min; proteinuria up to 2 g/day; hypertension, and aortic dilatation (at the level of the ring, sinuses, and ascending aorta). In this family, one man aged 33 years had died from renal failure (DNA diagnosis was not made). The second 26-year-old man with the documented mutation in the clinical picture had proteinuria up to 2.5 g/l, persistent hematuria up to 100 red blood cells in the field of vision; a glomerular filtration rate decrease to 50 ml/min; sensorineural deafness, and cardiovascular abnormalities (dilatation of the left ventricle, ring, sinuses, and ascending aorta; aortic regurgitation, 2 cm; and stable hypertension).

The paper is devoted to the study of the rate of urinary tract abnormalities and kidney functions in children with mild, moderate, and severe connective tissue dysplasia. Severe connective tissue dysplasia was found to prevail in children with urinary tract abnormalities and to be characterized by a variety of urodynamic urinary tract abnormalities. Urinary system infection occurred equally frequently in both patient groups and its rate did not depend on the degree of the dysplasia. Some children with severe connective dysplasia were noted to have diminished renal filtration function. High-grade vesicoureteral reflux, tubular disorders as nocturias, and lowered urine osmolarity were more common in children with severe dysplasia. Hypertension was seen equally often in both patient groups, no matter what the degree of connective tissue dysplasia.

The specific features of a manifestation of genomic instability, as shown by the karyological indicators of the micronucleus test using buccal epithelial cells, were investigated. To assess an immune response to the mutagenic action of ionizing radiation, peripheral blood lymphocytes with the phenotypic characteristics of CD95+ subpopulations were quantified using flow-through cytofluorometry, by estimating the possible consequences of transferring the mutagenic effect of ionizing radiation to posterity. A total of 112 first- and second-generation patients residing in the areas polluted with radionuclide cesium-137 (more than 556 kBq/m2) were examined in the aspect of family. The data obtained in the investigation employing the models for individual families may anticipatorily consider certain cytogenetic indicators in second-generation children as predictors of carcinogenesis and the possibility of their transgenerational transfer in the generations of persons exposed to radiation.

The distribution of health groups among 14-year-old adolescents (n = 707) living since birth under the conditions of mid-latitudes (the towns of Kirov and Yaransk) and in the European North (the town of Ukhta and the settlement of Sedyu) was compared to determine the impact of anthropogenic pollution at different latitudes on this indicator. It was shown that the conditions of the European North in the absence of anthropogenic pollution failed to affect the number of 14-year-old boys and girls having health groups I, II, III, IV, and V. Anthropogenic pollution was found to decline the number of health group I adolescents living in the European North, without influencing this indicator in those dwelling at mid-latitudes, but, in spite of the latitude of their residence, to increase that of persons with health group II, without having an impact on the number of persons with health groups III, IV, and V.

The paper presents the experience of complementary feeding in children with manifestations of food allergy. The 4-day food rotation diet proposed by a group of authors from the Children’s Health Research Center (2009) for children 3 years of age and older has been used. The diet has been modified for babies of the first year of life who have manifestations of polyvalent food allergy, which could maximally extend the range of foods and minimize the clinical manifestations of food allergy.

It is concluded that to diversify the range of complementary foods, it is appropriate to use the 4-day food rotation diet designed by a group of authors from the Children’s Health Research Center; the first complementary foods should include specialized commercial infant formulas proven to be hypoallergenic.

Objective: to evaluate the physical development and health status of babies receiving goat’s milk-based formula “Bibikasha”.

Patients and methods. An open-label uncontrolled trial was conducted in 47 babies aged 5 to 6.5 months. Their weight and height changes, neuropsychological development, and the skin were assessed. The incidence of acute respiratory diseases, the manifestations of dyspepsia, and number of bowel movement a day were estimated; fecal macroscopy, microbiological examination, and complete blood count were carried out. The data were statistically processed using Statistica Advanced.

Results. At complementary feeding, there was constipation and hard stools in approximately 20% the infants in the study group and fecal opportunistic bacteria in 63.8%. When eating Bibikasha, the number of infants with opportunistic pathogenic bacteria reduced by 3 times and hard stools and constipation disappeared completely. During their follow-up, none of the babies developed anemia, acute respiratory or gastrointestinal diseases; weight gain rates and psychomotor development were age-appropriate, indicating their harmonious development.

Conclusion. Bibikasha used as a complementary food has a positive effect on a baby’s health and contributes to the prevention of nutrition-related diseases. Adding Bibikasha to the diet of infants with constipation normalizes their bowel function

The paper analyzes current clinical practice and researches dealing with diabetic neuropathy in children and adolescents. It shows the problems associated with the diagnosis of different types of diabetic neuropathy. The prevalence of its peripheral and cardiovascular types is characterized on the basis of the author’s investigations. Diagnostic criteria for its gastrointestinal type are proposed according to 24-hour pH-metry readings. Prospects for further investigations dealing with determination of diagnostic criteria, elaboration of unified approaches to interpreting the results of an investigation, and specification of indications and drugs for treatment are identified.

The paper highlights the principal items of use of decongestants in the treatment of rhinosinusitis in children. It analyzes the advantages and disadvantages of all classes of decongestants and their role in the pathogenetic therapy of rhinosinusitis. The role of complex vasoconstrictors including excipients (certain components of essential oils) is defined, by using Lasolvan®Rhino as an example. The efficient and timely therapy of acute rhinitis in children can considerably reduce the frequency of complications and improve a child’s health, helping restore normal eating and sleep. Since the earliest treatment is of the greatest importance in the therapy of acute rhinosinusitis, emphasis is placed on decongestants within the first days of the disease. The decongestant Lasolvan®Rhino (Boehringer Ingelheim) fully meets the goals of treatment for acute rhinosinusitis in children over 6 years of age as an effective, reliable, and safe agent, as evidenced by clinical trials.

In memory of teacher Vyacheslav Aleksandrovich Tobolin.

A review of the monograph “Orphan lung diseases in children” by N.N. Rozinova and Yu.L. Mizernistky

Congress «Pediatric cardiology 2016».