An extensive meta-analysis of the world literature showed that congenital heart diseases were registered at a rate of 6 per 1,000 live births in 1930—1934 with its increase up to 9,1 per 1,000 after 1995. According to various estimates, the rate of congenital heart diseases was 4 to 10 per 1,000 births after 2000. However, the true prevalence of the above defects may be much higher. Most authors are in agreement that the rate of congenital heart disease varies between 19 to 75 per 1,000 live births; serious anomalies being registered at a rate of 19,1—23,9 per 1,000 births. Many cardiologists do not consider mild defects to be diseases; however, this statement calls for further investigation. Fetal echocardiography and genetic studies are of great importance in estimating the prevalence of congenital heart disease. Tables of chromosomal and monogenic syndromes associated with cardiac malformations are given.

The intracardiac echogenic focus, a small rounded hyperechogenic structure, which is found in the fetal cardiac ventricles in the region of their papillary muscles or chordae tendineae, executes motions together with the atrioventricular valves, so is colourfully named «a golf ball». It is more frequently visualized by prenatal ultrasound screening for congenital abnormalities in the early fetal period. This finding is assigned to ultrasound markers, the clinical value of which has remained debatable and ambiguous over the past 20 years. This paper gives the current views of the causes and mechanisms of intracardiac echogenic foci, the time course of their changes during intrauterine fetal development and after a baby's birth.

Necrottzing enterocolitis remains a major cause of morbidity and mortality among low and extremely low birth weight premature infants. There has been a trend within recent years to increase the percentage of newborn babies with necrotizing enterocolitis, which is associated with the intensive development of neonatology and intensive care. It is extremely difficult to make the diagnosis in low birth weight infants in the early stages of the disease. To study actively the most important diagnostic factors in the early stages of the disease is one of the first tasks of neonatologists. X-ray examination is the most reliable diagnostic method when the clinical manifestations necrotizing enterocolitis appear. When the disease develops its surgical stage, mortality rate increases dramatically among extremely low birth weight premature infants. Choice of the best surgical treatment in these babies remains an important challenge faced by pediatric surgeons.

Kawasaki syndrome is an acute systemic vasculitis of unknown etiology, which mainly affects children within the first 5 years of fife. At the present time, Kawasaki syndrome is recognized to be a leading cause of acquired organic heart diseases in children, which may result in coronary heart disease, myocardial infarction, and sudden death in children and young people. Most complications are associated with the cardiovascular system, with coronary artery changes in particular. Transthoracic echocardiography, which, besides coronary artery assessment, makes it possible to evaluate right and left ventricular systolic and diastolic functions and to study the cardiac valves and changes in pericardial effusion, is a major technique in Kawasaki syndrome. The paper outlines an update on the epidemiology, etiology, pathogenetic mechanisms, and pathomorphology of Kawasaki syndrome and considers possible coronary and noncoronary changes, outcomes, and clinical manifestations.

Kawasaki syndrome is an acute systemic disease. It is clinically characterized by fever, changes in the mucous membranes, skin, lymph nodes, and possible involvement of coronary and other visceral arteries. The disease is encountered mainly in children less than 5 years of age. In the patients aged less than 6 months it occurs less frequently and may be severe. These babies are at high risk for coronary artery aneurysms. The lack of alertness to the possible development of Kawasaki syndrome at this age delays treatment and worsens prognosis. The Kawasaki syndrome should be considered as a possible cause of prolonged fever even in youngest infants. High fever of unclear genesis in a baby within the first months and 7 days or more of life is an indication for echocardiographic examination of the coronary arteries. The presented review analyzes the studies conducted in different years in the USA, Canada, Korea, and on the specific features of the disease in infants less than 6 months, including in neonates.

The paper discusses the current literature data and the authors' ones on the accurate diagnosis of mitral valve prolapse and its role in an athletic population. A comparative analysis shows a substantial (as high as 4—9-fold) reduction in the average rate of both the prolapse and its complications when diagnostic criteria are changed. Among 500 athletes referred for echocardiographic examination in the past 2 years, the rate of mitral valve prolapse has been only 0,2%. Assessment of the role of the prolapse indicates that the valve regurgitation that can increase during exercise is of basic value; in this connection, it is expedient to carry out exercise echocardiography tests.

The review of the literature shows the results of current researches dealing with the role of ghrelin in metabolic processes. Ghrelin stimulates food intake and is involved in the regulation of energy homeostasis, carbohydrate metabolism, body weight, which allows this drug-based therapeutic vaccines to be further synthesized. The agent acts directly on the cardiovascular system and blood pressure. Numerous effects of the hormone on the function of the gastrointestinal tract are indicated. The hormone has a potent anti-inflammatory effect. Its action on learning processes and its possible involvement in the pathogenesis of depression and neuro-degenerative diseases are described.

Nursing outcomes were evaluated in 110 extremely low birth weight infants to study the characteristics of a maternal reproductive history, the conditions of fetal development, and their early neonatal period. The babies from women with a history of miscarriage were significantly frequently recorded to have severe bronchopulmonary dysplasia, generalized pyoseptic diseases, CNS lesions as grades II—III intraventricular bleeding.

Seventy children aged 7 to 17 years with hypertrophic cardiomyopathy (HCM) were examined; among them there were 11 syncope patients and 5 presyncope patients. The screening program included standard electrocardiography (ECG), Doppler echocardiogra-phy, 24-hour Holter ECG monitoring, and an incremental exercise testing (Bruce treadmill test). The markers of myocardial electrical instability were determined. In the children with HCM, syncope was established to be heterogeneous; it had an arrhythmogenic origin and, in most cases, occurred in the presence of tachyarrhythmia (44%) or bradyarrythmia (25%); its vasovagal genesis was probable in one third of the examinees. The children with syncope were typified by the asymmetric, obstructive form of HCM, at the same tone there was most commonly left ventricular hypertrophy concurrent with left atrial enlargement. 24-hour Holter monitoring showed that bradycardia was prevalent in the patients, 3 patients were found to have more than 2-second cardiac rhythm pauses caused by second-degree atrioventricular block in 1 case or by sick sinus syndrome in 2. Nonsustained ventricular tachycardia was noted in two patients. The children with syncope were typified by the signs of myocardial electrical instability as a reduction in the early phase of heart rate turbulence and by impaired QT/RR interval adaptation as hyperadaptation. The paper presents the developed management tactics for children with syncope and indications for the implantation of a cardioverter defibrillator, a pacemaker, or an ECG loop recorder.

The diagnosis and therapy of syncope in children are an urgent health problem. The paper describes a clinical case of long Q— Tinter-val and its clinical picture, diagnosis, and treatment. Current approaches using p-blockers in combination with cardiotrophic therapy are shown to improve the prognosis of the disease and to open new vistas for the treatment of these patients.

The diagnosis and treatment of life-threatening arrhythmias after surgical correction of congenital heart diseases is one the urgent problems in pediatrlc cardiology. The paper describes the clinical picture, diagnosis, and therapy in a patent with transient complete atrioventricular block after open heart surgery. A protracted Morgagni—Adams—Stokes attack is one of the causes of sudden cardiac death. The occurrence of even single syncopes in patients after surgery for congenital heart disease requires that the arrhythmogenic nature of the attack should be ruled out for the timely and adequate therapy including the implantation of antiarrhythmic devices.

Autonomic nervous system dysregulation is one of the leading components in the pathogenesis of neurogenic bladder dysfunction. These disorders lead to diverse changes in the functions of the sympathetic and parasympathetic systems with disordered release of mediators (norepinephrine, acetylcholine), hormones of the adrenal cortex and other endocrine glands, a number of biologically active substances (polypeptides, prostaglandins), as well as to the impaired sensitivity of vascular a- and p-adrenoceptors. Children with dysuria concurrently develop visceral, CNS, and circulatory system dysfunctions and metabolic disturbances. The paper describes the clinical trial of children with overactive bladder, which demonstrates the autonomic tone in these patients (и=44). The findings point to the important involvement of the autonomic nervous system in the pathogenesis of the disease and provide a rationale for the incorporation of vegetotropic drugs normalizing the autonomic nervous system into the combination therapy of overactive bladder.

Objective: to identify risk factors of frequent relapses nephrotic syndrome in children to choose an optimal treatment policy. Materials and methods. Sixty-six case histories of children with steroid-sensitive nephrotic syndrome were retrospectively analyzed. We investigated relationship between frequency of relapses and clinical manifestations of the disease, namely: gender; age; duration of steroid therapy; time to achieve remission during steroid therapy; time to develop the first relapse of nephrotic syndrome. Results. The age at the onset of nephrotic syndrome younger than 2 years; therapy with daily oral prednisolone (2 ing/kg/day) less than 4 weeks; the total duration of steroid therapy at the onset of disease less than 3 months; the time to achieve remission after initiation of steroid therapy more than 10 days and the time to develop the first relapse less than 5 months after manifestation of nephrotic syndrome are the risk factors of the frequently relapsing disease. Conclusion. The found risk factors of frequent relapses of nephrotic syndrome in children proves the need for adequate steroid therapy in the onset of disease and indication for appointment steroid-sparing agents at the early date to prevent side effects of prednisolone therapy.

The purpose of the study was to determine the pattern of neuropsychic developmental disorders in children and adolescents with progressive muscular dystrophies (и= 19) and congenital structural myopathies (я=10). The syndrome method developed by A.R. Luria was used to analyze the higher mental functions. The patients were found to have impairments in visual-spatial function, somatosen-sory gnosis, and tactile sensitivity. In the children and adolescents with progressive muscular dystrophies, neuropsychological deficit is higher than that in the patients with congenital structural myopathies. The pattern of higher mental dysfunctions will be able to predict the future mental development of a child and to choose adequate methods of psycho-pedagogical correction.

Attention has been repeatedly drawn to the fact that persistent thymic hyperplasia (ICD-10 E32.0) is concurrent with morphological and/or functional disturbances of the nervous, endocrine, and immune systems, with various malformations and a number of other abnormalities in children. A retrospective controlled study was conducted through computer monitoring of the screenings of children of decreed ages. Children diagnosed with thymic enlargement have been found to have significantly more (functional and chronic) diseases and some conditions and nosological entities than the comparison group. All these abnormalities, as persistent thymic hyperplasia, are characterized by the presence of aggravating factors during ontogenesis. Thus, the association of persistent thymic hyperplasia with other abnormalities is due to the influence of similar factors, i.e. the fact that the increase in the volume and weight of the thymus above the limiting age values with maintenance of its normal histoarchitectonics arises from the joint action of genetic and environmental factors must not be ruled out.

The paper describes the clinical use of a phosphodiesterase 5 inhibitor (sildenafll) in a patient with primary pulmonary hypertension during a long-term controlled follow-up. The 6-year use of sildenafll as monotherapy for pulmonary hypertension had a significant positive effect as a decrease in the functional class of pulmonary hypertension from 3 to 1, an increase in exercise tolerance, improvements in echocardiographic parameters and biochemical markers (brain natriuretic peptide), and a rise in oxygen uptake during cardiorespiratory testing.

The article presents current approaches to diagnosing and treating polyarteritis nodosa in children. This clinical case demonstrates diagnostic difficulties associated with the nonspeciflcity of initial symptoms of the disease, with the polymorphism of its clinical manifestations, and with the absence of specific morphological and aortographic markers. The diagnosis was made by excluding various rheumatic, infectious, and neoplastic diseases. The peculiarity of this clinical case was the severe course of classical polyarteritis nodosa accompanied by cerebral vascular crises in an infant with a compromised family history.

Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an autoimmune central nervous system disease mediated by antibodies to certain subunit of the N-methyl-D-aspartate receptor and characterized by the development of severe psychiatric and neurological disorders in previously healthy children. The paper describes a clinical case in an 8-year-old female patient with this disease presenting with seizures, psychiatric symptoms and behavioral problems. Then the disease progressed to dyskinesis and memory and speech disorders. The final diagnosis of anti-NMDA receptor encephalitis was made after identifying anti-NMDA receptor antibodies in the cerebral spinal fluid.

The paper describes the unusual location of cavernous transformation (cavernoma) in the portal vein, which has caused a clinical and hematologic picture of Kazabach—Merritt syndrome and portal hypertension in a 7-year-old boy.

Cow's milk protein allergy is prominent in the pathogenesis of allergic diseases in infants during the first year of life. The allergenic properties of protein are diminished by its hydrolysis. The purpose of this open-label muMcenter prospective study was to evaluate the efficiency of nutritional therapy for cow's milk protein intolerance with Nutrilon® Amino Acids, Nutrilon® Pepti Allergy, and Nutrilon® Pepti Gastro in 59 formula-fed infants with the severe and moderate manifestations of food allergy during the first year of life. At the first stage of the study (42 infants) by the end of week 2 of nutritional therapy, there was a full achievement of positive results in 24 (57%) patients and significant and moderate improvements in the skin process in 12 (28%) and 6 (15%) patients, respectively. At the second stage of the study, the newly included babies receiving a fully hydrolyzed formula showed the following results: a mil achievement of positive results in 16 (50%) patients and significant and moderate improvements in the skin process in 9 (30%) and 6 (20%), respectively. Thus, the study demonstrated the high efficacy and good tolerability of the formulas Nutrilon® Amino Acids, Nutrilon® Pepti Allergy, and Nutrilon® Pepti Gastro, by rapidly and persistently achieving the result.

The clinical efficacy of adapted Nanny 1 and 2 formulas containing prebiotics in the diet of infants with functional indigestion during the first year of life was evaluated. A total of 120 babies during the first year of fife were followed up. Of them, 27 patients within the first 5 months of life received the formula Nanny 1 with prebiotics, which was replaced by Nanny 2 with prebiotics in 33 infants during 5,5—6 months of fife (a study group). A comparison group consisted of 60 formula-fed infants of the same age who switched to the formula Nanny classic without prebiotics. During the use of Nanny goat's milk based formulas for 1 to 3 months, there was a reduction or cessation of the symptoms of functional ingestion, improvement in stool pattern, and a decline in the number of infants with disordered intestinal microflora. The values of secretory IgA increased and the level of C-reactive protein decreased in the coprofiltrates, suggesting that there was increased higher local intestinal immunity and reduced inflammation. The most pronounced positive effect was observed in the babies who were fed the formulas containing prebiotics. Thus, Nanny 1 and 2 formulas with prebiotics for feeding babies during the first year of life show good tolerability, relieve the symptoms of functional indigestion, and prevent enteric dysbiosis and chronic eating disorders.

The paper considers how to organize properly balanced feeding in infants during the first year of life, by using additional foods. It highlights current approaches to using the latter in the babies and provides a rationale for the tune of giving additional foods, by taking into account the readiness of an infant to eat new foods and the need to incorporate micronutrients for the prevention of anemia, rickets, and food allergy. The benefits of using commercial products are discussed.