LEAD ARTICLE
The article gives basic theories of the pathogenesis of atherosclerosis, including inflammatory, cholesterol, lipid, lipoprotein, iron ones, as a result of metabolic syndrome, oxidative stress. In spite of carefully and deeply developed and ongoing elaborated pathogenesis theories, the etiological factors of atherosclerosis remain unknown so far. The age-related aspect of the disease is discussed; atherosclerosis is considered to be a childhood-onset disease that manifests itself at a later age. The authors propose an experimental and clinical evidence-based concept of the common etiology of syndromes of atherosclerosis, namely: the body's endogenous intoxication that is permanent or periodically progressive may be a primary cause of altered conformation of different protein molecules with their higher ability to adsorb the trace elements consolidating the structural changes. This change of proteins diminishes their functions and determines their antigenic properties, which is attended by the development of different pathogenic components in relation to the body's individual features.
REVIEWS
The review of modern literature covers main problems in the use of non-invasive ventilation in preterm infants. The advantages and disadvantages of various devices and methods are identified. The criteria for the efficiency of non-invasive ventilation and the predictors of its failure are presented. At the moment, non-invasive ventilation methods are increasingly being used in preterm infants at hospitals at any level and even in the smallest patients.
The paper gives the data available in the literature on the current classification, genealogy, clinical manifestations, diagnosis, treatment, and prevention of mucopolysaccharidosis, one of the most common nosological entities of lysosomal storage diseases. Particular emphasis is laid on cardiovascular pathology in this group of diseases. Heart damage is ascertained to be one of the cardinal signs of mucopolysaccharides frequently leading to rather early fatal outcomes. Cardiac pathology is notified in all types of muco-polysaccharidoses; however, it is most important for patients with three clinical variants of Hurler syndrome, Hunter syndrome, and Maroteaux-Lamy syndrome. According to the data available in the literature, the characteristic signs of cardiovascular system damage in mucopolysaccharides should be considered to be: thickening of the valves with the development of their dysfunction (the severity of left-sided valve lesion being more pronounced), myocardial hypertrophy, conduction disturbance, coronary artery lesion, and arterial hypertension. Many investigators emphasize difficulties in clinically and functionally examining the cardiovascular system in patients with mucopolysaccharides, which is due to physical and intellectual limitations in the patients and to a gradual progression of symptoms. Medical and surgical methods were used to treat cardiovascular disease in mucopolysaccharides. Enzyme replacement therapy and hematopoietic stem cell transplantation have become much more topical in the past years. These methods may stop disease progression and favor regression of ventricular hypertrophy and stabilization of left ventricular function.
The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.
Down's syndrome is one of the most common genetic diseases. Recent studies have indicated that gene dosage effects and imbalance of gene expression underlie the disease. There is a review of the data available in the literature that in Down's syndrome there is oxidative stress that gives rise to oxidative injury and mitochondrial DNA dysfunction and seemingly determines the severity of neurodegenerative disorders. The role of coenzyme Qlo in bioenergy metabolism and the possibility of its use to correct metabolic and psychoneurological disorders in children with Down's syndrome are discussed.
In accordance with the current definition proposed by the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) in 2012, celiac disease is regarded as an immune-mediated systemic disease caused by gluten and respective prola-mins in genetically predisposed people. Detailed studies of the pathogenesis of gluten enteropathy provided the basis for investigating the possible causes of the common concurrence of celiac disease with a number of autoimmune and endocrine diseases. The possible reasons for the association are considered to be common genetic markers in the patients, a cross reaction of the autoantibodies formed in celiac disease and activated T lymphocytes with the body's intrinsic antigens, as well as the systemic action of proinflam-matory cytokines. Although many issues of pathophysiology remain a matter of debate, the fact that patients suffering from a number of diseases form a group at risk for celiac disease and require a careful follow-up and examination for timely diagnosis and use of gluten-free diet is currently beyond question.
PERINATOLOGY AND NEONATOLOGY
Pneumonia is the most common intercurrent disease in babies with congenital heart disease in the preoperative period and a reason for the forced delay of cardiac surgery. The investigation has proven that the course of pneumonia may be predicted in babies with heart defects to choose the optimal algorithm for diagnosis and treatment in order to reduce a preoperative period.
The aim of the study was to identify kidney dysfunction in infants born prematurely to mothers with preeclampsia. Forty-eight preterm infants, including 15 babies born to mothers with preeclampsia (a study group), 13 neonates whose mothers had complicated pregnancy without preeclampsia (a comparison group), and 15 apparently healthy infants (a control group) were examined. To evaluate renal tubular function, the indicators, namely: kidney injury molecule-1 (ЮМ-1) and neutrophil gelatmase-associated lipokalin-2 (NGAL) were determined on 1—3 and 7—10 days of their life. Glomerular filtration rate was estimated to assess the performance of the glomerular apparatus. The study group was found to have the high values of tubular injury markers on 1 to 7—10 days of life, which significantly differed from those in the control and comparison groups (/КО,05). The glomerular filtration rate reflecting the capacity of the glomerular apparatus in the infants born to preeclamptic mothers was lower than that in the control group, but similar to that in the comparison group with a tendency towards normalization by the end of the early neonatal period. Thus, glomerular injury was short-term and abolished during adequate therapy whereas the high urinary levels of KJM-1 and NGAL from the first to 10th days of life confirm persistent tubular injury in the infants born to preeclamptic mothers.
The statistical model could consider the influence of specific maternal psychoemotional and personality factors on a newborn with cerebral ischemia and develop a procedure to prevent negative consequences of postpartum depression in the mother-baby system.
The analysis of echocardiography results in 394 fetuses and 570 neonates showed the high accuracy of prenatal diagnosis of congenital heart disease (CHD) in a specialized cardiac surgery facihty. The accuracy of critical CHD identification at the unspeciahzed stage of fetal diagnosis was 50%. Heart defects, such as aortic coarctation, interrupted aortic arch, and total anomalous pulmonary venous drainage, turned out to be most difficult for prenatal diagnosis (at all stages). There were no problems in the prenatal diagnosis of the following defects: tetralogy of Fallot, all types of single ventricle defect, and atrioventricular septal defect. The discordances between the diagnosis of CHD by fetal echocardiography at an obstetric stage and that in a specialized cardiology facihty were mostly due to the inaccurate description of all components of complex heart defects.
GASTROENTEROLOGY
То analyze medical care for children with gastroenterological diseases in the Transbaikal Territory, the author studied their incidence from the referral rates of children and adolescents, by examining the statistical materials of the Medical Information and Analytical Center of the Transbaikal Territory. The 2006—2012 morbidity rates per 1000 children were calculated. In the children, digestive diseases were found to rank second, amounting to 8,5% of all pathology cases. There was a tendency for the incidence of gastrointestinal diseases to decrease. However, it continues to remain higher in the Transbaikal Territory than in the entire country. For all the years, gallbladder diseases headed the list of the above diseases, gastritis and duodenitis ranked next, non-infectious enterocolitis occupied the third place. The interpretation of the results of additional studies and the issues of treatment and prevention are discussed. It is concluded that there is a need for the early prevention of gastroenterological diseases when their risk factors form. Increased attention to gastroenterological diseases in children will be a prerequisite for reducing their incidence in an adult population.
CARDIOLOGY
The article provides a comparative analysis of the antihypertensive and cardioprotective effects of magnerot® on pathological cardiac remodeling and repolarization in children with sustained hypertension complicated by left ventricular hypertrophy. Stress testing before and after combined therapy with angiotensin-converting enzyme inhibitors and magnerot® revealed higher exercise tolerance and a normalized blood pressure response in hypertensive children with left ventricular hypertrophy. Moreover, there was a reduction in the spatial dispersion of repolarization. These results substantiate the need to incorporate magnerot® in combination with angiotensin-converting enzyme inhibitors into therapy for patients with hypertension complicated with left ventricular hypertrophy.
It is frequently difficult to establish the pathogenetic mechanism of faints in patients with sinus node dysfunction due to the similar clinical manifestations of cardioinhibitory reflex syncope and Morgagni—Adams—Stokes syncopal episodes; to the intermittent course of sick sinus syndrome; to no correlation between the clinical manifestations and duration of asystole at the moment of the episode. The described clinical case demonstrates a diagnostic search for the mechanism of syncopal states running with asystole in a teenager with sinus node dysfunction. A long-term (48-month) follow-up in a female patient repeatedly documented asystoles during provoked syncopal and presyncopal states, which could ascertain the reflex genesis of syncope (its cardioinhibitory type with asystole). A 12400-ms asystole episode recorded with ECG loop recorders (REVEAL) was single, accompanied by no clinical manifestations and therefore it had no impact on further treatment policy.
INFECTIOUS DISEASES
Childhood encephahtis is as high as 19—20% in the structure of morbidity of neuroinfections and a high risk for neurological deficit. The study was undertaken to delineate the clinical, etiological, and topical characteristics of encephahtis in children from the 10-year data of the Research Institute of Childhood Infections. The clinical presentation, etiology, and magnetic resonance imaging results were analyzed in 302 children aged from 1 month to 18 years with encephahtis. The pattern of encephalitides was ascertained to present as leukoencephalitis (75%), polioencephalitis (17%), and panencephahtis (8%). Viral encephahtis was found in 76% of the children whereas bacterial encephahtis was seen in 8,7% and mainly associated with B. burgdorferi s.l. Leukoencephalitis was specified to be related to herpesvfruses, more commonly to its types 3, 4, and 6 in 78% of cases. In 82% of cases, the etiology of panencephahtis was associated with congenital viral infection, among which cytomegalia amounted to almost one third and polioencephalitis was caused by tick-borne encephahtis virus in 45% of cases. Leukoencephalitis was characterized by impaired sensitivity and cerebel-lar symptomatology with an outcome to recovery in 78% of cases. Polioencephalitis showed a preponderance of unconsciousness and convulsions whereas panencephahtis exhibited movement disorders and cognitive impairments with an outcome to cognitive or neurological deficit in 45—77% of cases. Thus, the topical characteristics of encephahtis in children determine its clinical presentation and depend on age and etiology. The established correlation between these parameters makes it possible to improve the early diagnosis and prognosis of the disease.
The performed investigations established that children with intestinal parasitic diseases were more prone to various diseases than those without the latter. In particular, in the last 2 years, there was a total of 78,2+1,5% and 36,4+2,0 cases in the former and the latter, respectively (г=16,72; /КО,001). Overall, an average of 0,78+0,11 cases was per child with intestinal parasitic diseases with 2,82+0,07 absences from school. Intestinal parasitic diseases are an urgent sociomedical problem that is primarily due to high mortality rates in children, to lower academic achievements and physical activity.
TO THE PRACTITIONER ASSISTANCE
The paper gives data on the possibility of expanding the assortment of foodstuffs for pregnant women with commercial baby foods.
То optimize the feeding of preterm infants is now one of the priority areas in neonatology and pediatrics because neonatal malnutrition has a negative impact on the health and development of a preterm infant, including on those at a later age. Current recommendations for the enteral feeding of preterm infants are based on the knowledge of their anatomic and physiological features and the need to prevent a menacing disease, such as necrotizing enterocolitis. The feeding of preterm infants at any gestational age with their mother's native breast milk is most optimal. Recommendations for breast milk enrichment are warranted for preterm infants with higher nutrient requirements. The paper presents the differential algorithms and regimens for the feeding of preterm infants with both breast milk and milk formulas depending on many factors, such as gestational age, birth weight, and clinical status.
ANNIVERSARIES
НЕФРОЛОГИЯ
Based on their studies and the data available in the literature, the authors consider current views on hereditary nephropathies running with hematuria, such as Alport syndrome, thin basement membrane disease, C3 glomerulonephritis, etc.).
ISSN 2500-2228 (Online)