The paper assesses global achievements in childhood pulmonology and prospects for their introduction into medical practice. It determines the contribution of developments by Russian scientists, the Moscow Research Institute of Pediatrics and Pediatric Surgery, Ministry of Health of Russia, in particular, to the elaboration and introduction of these promising research areas. The importance of not only theoretical, but also applied researches is emphasized and their priorities are arranged.

The review presents the results of an analysis of the data available in the Russian and foreign literature, which show that severe gestosis (preeclampsia) is a main cause of perinatal morbidity and mortality. Severe gestosis leads to impaired fetal growth and development, premature births, the development of perinatal hypoxic lesions of the central nervous, cardiovascular, endocrine, immune, respiratory, and other systems, which adversely affects the health status of children in their later life. Women with a history of preeclampsia or eclampsia are further at increased risk for cardiovascular (atherosclerosis, coronary heart disease, peripheral arterial diseases, and hypertension), cerebrovascular diseases, abnormalities of the kidney and organs of vision, diabetes, and memory impairments.

The paper analyses recent publications on the clinical manifestations and diagnosis of mitochondrial diseases caused by defects in nuclear genes. A scientific analysis included about 100 genes. According to the encoded protein and its function, the author has identified 9 gene groups that affect the processes of cellular bioenergy. By the time of their manifestation, the diseases were divided into groups: those of early childhood (including neonatality), childhood, adolescence, and adulthood. Attention is drawn to difficulties to identify some forms of the diseases in view of the clinical polymorphism of manifestations of mutations in individual genes and, at the same tone, many similarities between clinical symptom complexes caused by different enzyme and gene defects. There are additional criteria for the differential diagnosis of the diseases: 3-methylglutaconic aciduria, depletions and multiple depletions of mitochondrial DNA. It is concluded that it is necessary to more extensively introduce the whole-exome sequencing test that can reveal not only common, but also rare gene mutations in nuclear DNA. Gene defect identification permits medical genetic counselling and prevention of the spread of severe pathology in the family.

Cell energy metabolic disorders, the basis for which is mitochondrial insufficiency caused by mitochondrial DNA (mtDNA) point mutations, give rise to a broad spectrum of clinical manifestations so the purpose of this review is to analyze the recent publications on the relationship of mtDNA point mutations to mitochondrial diseases, which unveil the importance of development of molecular diagnosis. The presence of A3243G, T3271C, T3291C, C3256T, A8344G, G8356A, A3260G, СЗЗОЗТ, and A4300Gmutations in mtDNA may suggest that there are multiorgan dysfunctions and multisystem disorders, the clinical signs and symptoms of which can vary with time, which emphasizes the importance of comprehensive genetic studies if the mitochondrial disease is assumed to be clinical.

The paper presents the current views regarding the concept of unity of pathological changes in the skeletal and cardiac muscles in children with inherited neuromuscular diseases: myodystrophies, spinal amyotrophies, congenital myopathies, myonbrillar myopa-thies, metabolic myopathies, myotonia, polyneuropathies, and etc.

Congenital adrenal hyperplasia is a group of inherited diseases associated with a variety of adrenal enzymatic defects leading to disorders of sex development. The diseases are characterized by a triad of essential characteristics: decreased cortisol production, high blood ACTH levels, and bilateral adrenal hyperplasia. The review analyzes the diagnosis of congenital adrenal hyperplasia, as well as data on menstrual cycle dysfunction giving rise to impaired reproductive health in girls.

The problem of obesity is currently being widely discussed by various specialists. The literature dealing with this problem places a considerable emphasis on investigations of lipid metabolism disorders that occupy a prominent place in the genesis of cardiovascular diseases in the populations of most countries. The special role of long chain polyunsaturated fatty acids that are major functional components of brain membrane structure phospholipids, retinal photoreceptors is emphasized. Pediatricians pay great attention to the nutrition of infants, by adjusting adapted milk-based formulas for their adequate growth and development. At the same time, no consideration is given to the possibility of correcting obesity-associated problems with appropriate adapted milk-based formulas in older infants. However, dietary recommendations for older infants with obesity and overweight should be developed not only in the context of optimization of a qualitative and quantitative diet that ensures the needs of the growing organism at the present moment, but should take into account the prevention of cardiovascular diseases and other complications of obesity in the future.

The purpose of the investigation was to determine the umbilical cord venous blood level of glucose in full-term newborns and its relationship to the mode of delivery. The investigation included 102 full-term newborn infants, including 33 and 69 babies born via cesar-ean and vaginal delivery, respectively. Umbilical cord serum glucose levels were determined by the glucose oxidase test using a Sap-phire-400 biochemical analyzer. In healthy full-term newborns, the mean umbilical cord blood glucose levels were 4,29±0,88 mmol/1 (minimum, 2,9 mmol/1 and maximum, 5,9 mmol/1). In the babies born via cesarean delivery, the umbilical cord blood concentration of glucose was ascertained to be significantly lower than in those born vaginally (3,84+0,71 mmol/1 versus 4,51+0,87 mmol/1; /><0,0001). Abdominal delivery can be apparently considered to be a risk factor for hypoglycemia in neonatal infants.

The paper gives the results of an investigation of cardiac functional indexes in newborns with fetal growth retardation syndrome in the early neonatal period. The babies with fetal growth retardation syndrome are shown to have arterial hypotension. The characteristic feature of this contingent of infants is heart chamber remodeling followed by lower left ventricular contractile function.

The paper gives the results of clinical and laboratory examinations in 164 newborns of different gestational ages with moderate and severe hypoxic (central nervous system) injury and in 25 healthy infants from a control group. In the early neonatal period, depending on the severity of prior hypoxia, endothelial dysfunction of the neonates was characterized by a shift of endocrine activity toward the formation of vasoconstrictors, high serum vascular endothelial growth factor (VEGF) levels, and a thrombogenic tendency of the hemostatic system with inadequate hemostatic properties of a blood clot, as evidenced by piezoelectric blood viscometry. At one month of life, the newborns of the study groups were recorded to have reduced serum activities of endothelin-1, von WUlebrand factor, and VEGF. Hemostatic parameters became normal in infants with moderate hypoxic CNS injury by the end of the neonatal period while the inadequate hemostatic properties of a blood clot with enhanced activity of the fibrinolytic system were retained in those with severe hypoxic CNS activity.

Objective: to determine the levels pro- and anti-inflammatory mediators in children with perinatal pathology over time during an early adaptation period. The results of examination were analyzed in 53 newborn infants with perinatal pathology, including 18 babies with intrauterine infection, 20 with perinatal central nervous system (CNS) lesion, and 15 with birth asphyxia and no infectious or neurological diseases. A control group consisted of 30 healthy newborn infants with the physiological course of the early adaptation period. Enzyme immunoassay was used to determine the umbilical cord and peripheral blood levels of intercellular communication mediators (pro- and anti-inflammatory cytokines, acute-phase proteins) on 5—7 days of life. The reduction in interleuMn (IL)-ip, tumor necrosis factor-a (TNF-a), and IL-10 levels, which was associated with the elevated umbilical cord blood concentrations of interferon-Y (IFN-y) and IL-6, was ascertained to be a result of neonatal hypoxic changes. The distinctive characteristic of the cytokine profile in the infants with intrauterine infection is the detection of birth hypercytokinemia (IL-6 and IL-8 levels of more than 70 pg/ml) in conjunction with the high acute-phase protein concentration that is retained during the early adaptation period. The specific features of development of an immune response in the newborn infants with perinatal CNS injury are significant increases in the level of IL-8 within the first week of life and in the concentration of C-reactive protein on 5—7 days of life.

The paper deals with nosocomlal ventilator-associated pneumonia caused by gram-positive cocci in full-term and preterm newborns and with a trial of the clinical and microbiological efficacy of the antibiotic vancomycin. Thirteen full-term neonates and 46 preterm ones were examined. Most newborn infants (78—93%) were found to have monoinfection caused by gram-positive cocci, mainly Staphylococcus spp. and Enterococcus faecalis. Vancomycin was highly effective against the coccal microflora with the minimum inhibitory concentration (MIC) of <2 ug/ml against Staphylococcus spp. and Group В Streptococcus in 46 newborns, moderately effective with the MIC of 24 ug/ml, but s8 ug/ml against Enterococcus faecalis m9 infants and ineffective or lowly effective with the MIC of 216 ug/ml against Enterococcus faecium in 4 infants. However, secondary infection caused by the hospital strains of gram-negative microorganisms (predominantly Enterobacteriaceae and Pseudomonasaeruginosae) generally inducing mixed infection occurred in 41% of the infants on 5—7 day of the disease. In this connection, most children were given vancomycin in combination with beta-lactam antibiotics or amikacin.

The analysis of the incidence of congenital malformations (CMF) in the newborns of the city of Baku in 2000—2011 showed that in this period, 51 (4,8%) neonatal infants were born with different genitourinary abnormalities, which accounts for 1,77 per 10 000 births. In the period 2000 to 2011, the rate of babies born with genitourinary defects increased from 5 to 9%. Calculation of the incidence rate per 10,000 live births indicated that the rate was 1,8 in 2000, 3,6 in 2005, and 5,2 in 2011. The most common conditions were ascertained to be renal agenesis (29,5% of the newborn infants with genitourinary malformation) and true hermaphroditis (21,5%).

Objective: To determine the diagnostic value of insulin-like growth factor-1 (IGF-1) as a trophic marker in children with congenital heart disease (CHD) in the pre- and postoperative periods. The study included 149 CHD patients operated on at the N.F. Filatov City Children's Clinical Hospital Thirteen. The patients were divided into age groups: 1) 0 day to 12 months; 2) 1 to 3 years. IGF-I levels were measured in all the children before surgery, on days 2 and 5, and 6+0,5 months after surgery. Before surgery, 22,8% of the patients had a body weight Z-score of <—2SD; 11,4%, a height/age Z-score; 8,7%, a body mass index Z-score. IGF-I was lower for age in 53 (35,6%) children, <25 ng/ml (<25-65,0 ng/ml) in the patients of the first year of fife, and 68,65 ng/ml (<25—202,0 ng/ml) in those aged over 1 year. Six months after hospital discharge, there was a statistically significant (/K0,05) increase in IGF-I concentrations, which correlated with body mass index (r=0,501; /K0,01) and indicated a significant nutritional improvement in the examinees. Thus, the children with CHD showed the changed levels of IGF-I, which appeared as its low preoperative concentration in 40,7% of the patients of the first year of fife and in 23,9% of those aged 1 to 3 years. The follow-up study indicated that effective nutritional support caused 2,4-and 1,6-fold increases in this factor in the children aged 0—1 years and in those aged over 1 year, respectively, which correlated with increased body mass index. The findings give grounds to use this factor in the assessment of nutritional status in children with CHD.

Atopic dermatitis is a common chronic inflammatory disease in children. The disease onset and eruption progression most commonly occur at the age of less than 6 months and coincide with the timing of the first complementary foods. Complementary foods are given to infants with atopic dermatitis at the same tone as to healthy babies; however, there are significant differences in complementary feeding tactics. The paper discusses in detail principles for choosing the first complementary foods for infants with atopic dermatitis, rules for introducing of complementary foods, necessary patient preparation, and precautions. The given assumptions are strengthened by a clinical example.

The paper presents the results of statistical (sigma and percentile) analysis of height and weight in 434 boys and girls aged 14 years from the Voronezh Region in 2011 —2012. The girls' height was comparable with the regional indicators of physical development in the children of the Voronezh Region (1997—1999). The boys' height was characterized by a wide scatter of the obtained values; but there was an increase in the mean values as compared to the 1997—1999 data. Examinations of the boys and girls revealed a wide scatter in their weight with its increasing tendency. The performed examinations indicate the need for revision of the regional height and weight values for teenagers every ten years.

Recently, there have been many publications on the assessment of the health and development of children born after in vitro fertilization (IVF) in the families with fertility problems. The purpose of the investigation was to determine the level of intellectual development in the children born in the families with a history of miscarriage. The database consisted of 71 children born after lymphocy-totherapy in the families with reproductive losses. The children's intellectual development was evaluated by the method developed by D. Wechsler. The findings were compared with the testing data on 1,700 children of the same age in central Russia. According to the results obtained, the intellectual development of children born after lymphocytotherapy in the families with reproductive losses did not significantly differ from its population-based indicators. No association was found between the course of pregnancy and severe intelligence disorders in the examinees. The higher educational level of families with reproductive losses versus the population-based level can be inferred to be a factor for the better social adaptation of families who create better conditions for their children to develop.

Two hundred and fourteen children and teenagers aged 4 to 18 years were examined in a period of high incidence of influenza and acute respiratory viral infections (ARVI); of them 108 persons received a total of 4—5 cycloferon cycles in an age-adjusted dosage for 10 days for the complex prevention of influenza and ARVI. For treatment, 33 patients of the same age with influenza and ARVI had cycloferon in an age-adjusted dosage once daily for 10 days. Cycloferon used for prevention caused a 5,44-fold reduction in the incidence of influenza and ARVI; the protection ratio was 81,6%. Cycloferon used in the combination therapy of influenza and ARVI made them milder, reduced the time of their clinical manifestation and the incidence of complications, and normalized the indicators of the blood cytokine profile.

The paper presents the 2013 Institute's scientific achievements and developments made within the framework of the State Task in Initiative and Postgraduate Works. It describes the priority developments obtained using technologies in the diagnosis, treatment, prediction, and rehabilitation of childhood diseases with their efficiency being indicated. New progression markers and novel and improved therapeutic options are proposed for introduction; current approaches to preventing disease progression in children at different ages are substantiated.

The objective of the investigation was to study the ultrastructural morphology of muscle tissue mitochondria versus other clinical and laboratory indicators in 21 patients with congenital central core myopathy. The findings demonstrate that the sizes of mitochondria are correlated with the levels of lactate and pyruvate; however, the magnitude and direction of this correlation differ in the subsarco-lemmal and intermyofibrillar mitochondrial fractions. It is concluded that the baseline level of lactic acid in the blood of the patients depends on the number of mitochondria whereas the maturity of organelles largely provides a tissue response to elevated lactic acid levels. Our data are evidence in favor of an assumption that rounded mitochondria are less mature and effective in performing then-main functions. The increase in the number of ellipsoidal organelles in the subsarcolemmal region of muscle fibers is ascertained to be an important compensatory mechanism in nonmitochondrial muscle diseases. Thus, the investigation has established the role of changes in the number and sizes of mitochondria in tissue adaptation to the conditions of functional insufficiency. The found m