The article analyzes the recommended immunization schedule for premature babies. Due to the large number of routine medical disruptions this topic is extremely relevant in the Russian Federation today. Based on the modern scientific data the authors identified the difficulties of immunization of premature babies leading to an unreasonable delay in vaccination, also they assessed the safety and efficacy of vaccination of premature babies. They proved that the body of a premature baby develops a sufficient immune response to vaccine antigens, and therefore these children must be vaccinated without delay to prevent the severe course of infectious processes inherent in premature children. To reduce the controversy impeding timely immunization, there is a need for federal clinical guidelines for vaccination of premature babies with detailed explanations; some vaccines require special administration regimens, taking into account the reduced immunogenicity in this group.

Based on the analysis of foreign and domestic literature, the authors present the approaches to determining the phenotypes of bronchial obstructive syndrome in preschool children. They outline the data on the main foreign studies devoted to the peculiarities of the bronchial obstruction syndrome course depending on age and clinical outcome. They discuss the risk factors leading to the recurrent course of bronchial obstruction, tools for predicting bronchial asthma, and the choice of phenotype-dependent therapy strategy.

The calculated systolic, diastolic and mean pulmonary artery pressure, the ratio of the sizes of the right and left ventricles of the heart, the eccentricity index of the left ventricle, systolic excursion of the fibrous ring of the tricuspid valve, systolic movement of the myocardium at the level of the fibrous ring of the tricuspid valve and the area of the right atrium are the main echocardiographic indicators for assessing idiopathic and similar forms of pulmonary hypertension according to the current Russian, Eurasian and European guidelines for pulmonary hypertension in children and adults, as well as the recommendations of the 6th World Symposium on Pulmonary Hypertension. The article explores the possibilities and limitations of modern echocardiography in the diagnosis of pulmonary hypertension associated with congenital heart defects.

The concept of the steroid resistance in children with nephrotic syndrome, who were initially sensitive to steroids, has been known for a long time. Despite several foreign studies, this problem remains largely unexplored. The results of the presented review demonstrate a lack of information on many important aspects of resistant nephrotic syndrome. In particular, the true causes and pathogenesis of glucocorticosteroid resistance remain unknown, despite the long history of this problem in pediatric nephrology. On the one hand, such patients are clinically similar to the patients with steroid-resistant nephrotic syndrome, but at the same time they have a relatively favorable outcome in general. On the other hand, the ambiguous response to the steroid and immunosuppressive therapy hinders the development of a unified concept of the disease and pathogenetic therapy. Thus, an important task for future research is to find the reasons for the development of late (secondary) steroid resistance and, to develop and implement a personalized therapeutic approach to the management of children into clinical practice.

The health status of children under one year of age remains the most important problem of modern health care and prospectively determines the potential of the population. According to the data of the Federal statistical observation with the calculation of extensive, intensive indicators and rates of increase / decrease, the study of the main trends in the incidence of children in the first year of life in the Russian Federation for 2016–2019 revealed a decrease in the overall incidence of children in this age group. The morbidity structure remains unchanged and the first three places are occupied by the diseases of the respiratory system (48.0%), certain conditions of the perinatal period (10.21%), and diseases of the nervous system (9.35%).

The article discusses the current state of the problem of children physical development assessment in the light of the proposed documents of the Ministry of Health of the Russian Federation on preventive examinations of children involved in physical education and sports. However, the Russian Ministry of Health does not provide a normative and methodological document regulating the rules for the development of standards for physical development. The aim of our work is to develop standards for assessing the physical development of children of the first year of life in the region according to the method of regression scale, with the subsequent development of an algorithm for assessing physical development of a child by a pediatrician in accordance with the Ministry of Health of the Russian Federation. It is shown that the introduction into clinical practice of regional modified regression scale of body weight along with the height is preferable.

Early anemia of premature children is a characteristic disease of preterm infants, especially those with extremely low and very low body weight at birth. The study of new formation mechanisms will help to carry out preventive measures in a timely manner and prevent this pathology in the neonatal period.

Objective. To study insulin-like growth factor-1 and protein-3, which binds insulin-like growth factor, to predict early anemia and its severe variant in premature infants.

Characteristics of the children and research methods. The authors carried out the clinical and laboratory examination of 65 newborns of gestational age up to 32 weeks with a birth weight of less than 1500 g. The authors determined the content of insulin-like growth factor-1 and protein-3, which binds insulin-like growth factor in venous blood on the 3-5th day of life.

Results. The authors revealed new predictors of early anemia of premature children: the content of protein-3, which binds insulin-like growth factor, on the 3-5th day of life of 0.766 μg/ml or less, and the content of insulin-like growth factor of 25.9 ng/ml or less predict the formation of early anemia in premature and very preterm patients. The authors established the predictors of the severe variant of early anemia of premature children, requiring blood transfusion, namely: the content of protein-3, which binds insulin-like growth factor, taking into account the anthropometric parameters of the child at birth. They developed and implemented the methods for predicting early anemia and its severe variant in premature patients.

Conclusion. The results make it possible to timely carry out preventive measures in order to prevent the disease and improve the indicators of health and life quality in very premature patients.

In order to develop prognostic criteria for the development of necrotizing enterocolitis, the authors studied clinical and biochemical data of 108 premature infants born and nursed in the departments of the Republican Perinatal Center. The prognostic scale is based on the modification of the probabilistic Bayes method – the method of normalized intensive indicators with the calculation of prognostic, weight indices, normalized intensive and integrated indicators. Individual forecast of the development of necrotizing enterocolitis allows the development of treatment, prevention and rehabilitation measures.

Angelman syndrome is a genetic disorder characterized by mental retardation and severe speech delay, movement disorders and ataxia, dysmorphic features, and behavioral disorders. Angelman syndrome is caused by the loss of the 15q11.2-q13 region of chromosome 15 received from the mother, which leads to a violation of the expression of the UBE3A gene.

Purpose. To analyze clinical manifestations in children with Angelman syndrome to identify early-onset and characteristic clinical signs.

Characteristics of children and research methods. The study included 60 children. In all cases, Angelman syndrome was diagnosed on the basis of international clinical criteria and the results of genetic testing. The researchers used clinical, functional and molecular genetic research methods.

Results. 80-100% of children demonstrated delayed mental and motor development, lack of speech, affective behavior, ataxia, hand stereotypes, apraxia of hand movements, strabismus, sialorrhea. 72% of children had epileptic seizures; all patients (regardless of the presence / absence of epilepsy) had a pattern characteristic of Angelman syndrome on the electroencephalogram. Differential diagnosis was based on the gene / chromosomal syndromes characterized by similar clinical signs.

Conclusion. The combination of such most frequent, early clinical symptoms as difficulties in feeding, strabismus, impaired muscle tone, delayed motor and psycho-speech development, affective behavior with frequent laughter, and sleep disorders may indicate Angelman syndrome in a child.

Cystic fibrosis is a disease caused by mutations in a gene encoding CFTR-protein (Cystic Fibrosis Transmembrane conductance Regulator), located in the apical membrane of epithelial cells of the respiratory tract, intestines and pancreas. Defensins serve as important components of the innate human immune system, they play a key role in providing the first line of defense of a macroorganism against infection; they have high antimicrobial, antiviral, cytotoxic activity.

Objective. To determine the values of fecal β-defensin-2 in children with cystic fibrosis and to reveal the dependence of its level on the exocrine function of the pancreas and the severity of the patient’s condition.

Characteristics of children and research methods. The study included 57 children with cystic fibrosis, the average age was 20.93 ± 2.9 months. Cystic fibrosis was diagnosed on the basis of an increase in immunoreactive trypsin, sweat chlorides by Cook’s method (>60 meq / l). To assess the exocrine function of the pancreas the scientists determined the activity of fecal elastase. They evaluated the levels of fecal β-defensin-2 and calprotectin using a quantitative enzyme immunoassay.

Results. The levels of fecal β-defensin-2 were increased (108.2 ± 11.3 ng / ml) in all children under examination. The researchers found no correlation between the levels of fecal β-defensin-2 and fecal elastase. The level of fecal calprotectin was significantly higher in the group of children with cystic fibrosis as compared to the control group. There was a significant correlation between the levels of fecal calprotectin and fecal β-defensin-2 (r=0.57; p <0.05), however, no correlations were found between the levels of fecal β-defensin-2 and fecal elastase. The group of children with a severe course of the disease demonstrated an increase in the level of fecal β-defensin-2, fecal calprotectin significantly more frequent.

Conclusion. Children with cystic fibrosis demonstrated a significant increase in the concentration of β-defensin-2 as compared to the control group, which confirms the activation of the innate immune system of the intestinal mucosa. The researchers traced the relationship between high levels of fecal β-defensin-2 and the severity of the disease. The levels of fecal β-defensin-2 directly correlated with the concentration of fecal calprotectin and there was no correlation between the severity of pancreatic insufficiency and the concentration of fecal β-defensin-2.

Objective. To identify the features of the development of hormone dependence in children with hormone-sensitive nephrotic syndrome at the onset of the disease.

Characteristics of children and research methods. The authors carried out a retro- and prospective study with age assessment at the onset of the disease and first relapse in 118 children with hormone-sensitive nephrotic syndrome at the onset with a rarely and often recurrent course.

Results. The authors revealed a rarely and often recurrent course in 118 children with hormone-sensitive nephrotic syndrome at the onset of the disease, respectively, in 51 and 49% of cases with the development of hormone dependence (64%) and without (36%). They found statistically significant differences: the age of children at the onset of nephrotic syndrome, who developed hormone dependence and who did not develop, was 3.4 [2.2; 4.8] and 4.2 [3.0; 5.6] years accordingly (p<0.05); the age of children with nephrotic syndrome who developed hormone dependence and who did not develop hormone dependence by first relapse, was 3.8 [2.6; 5.1] and 5.7 [4.0; 7.4] years accordingly (p<0.001). The duration of the period until the first relapse after the onset in children with nephrotic syndrome, who developed hormone dependence and who did not develop hormone dependence, was 4 [4; 5] and 11 [7; 17] months, respectively (p<0.00001). Hormone dependence was revealed in 75 and 47% patients, respectively (p<0.05) among children with nephrotic syndrome who had the first relapse before 5 years of age and after 5 years of age.

Conclusion. The age of children at the onset and the first relapse of nephrotic syndrome, which developed hormone dependence, is significantly less than those without hormone dependence. Children with nephrotic syndrome who have had their first relapse before 5 years of age were more likely to become hormone-dependent.

The number of children newly infected with Mycobacterium tuberculosis continues to increase. Children from foci of tuberculosis infection are most often susceptible to primary infection. In recent years, the number of children diagnosed with latent tuberculosis infection has tended to increase. It is also important that the detection of active tuberculosis also continues to grow among such children.

Objective. To establish the role of risk factors and identify deviations in the quality of life in children with latent tuberculosis infection living in the foci of tuberculosis infection.

Characteristics of children and research methods. The researchers examined 110 children aged 0-14 years. They analyzed the risk factors for infection with Mycobacterium tuberculosis. The effectiveness of BCG vaccination was assessed by the presence and size of the scar. The quality of life was determined using the PedsQL questionnaire version 4.0 for children of 0-14 years old.

Results and discussion. There were much more children whose mothers suffered from tuberculosis in the family. There were much more children whose mothers released bacteria as compared to the children whose mothers did not excrete mycobacterium tuberculosis. The patients with an infiltrative form of pulmonary tuberculosis were the predominant source of infection in children. The children with diagnosed active tuberculosis with a history of latent tuberculosis infection were in close contact with a family member who had previously been in the penitentiary zone, and also had unsatisfactory living conditions.

Conclusion. The presence of an active form of tuberculosis in a child creates the preconditions for a comprehensive deterioration of all parameters of the life quality, while latent tuberculosis infection has an adverse effect only on certain aspects of the life quality. Psychosocial functioning is most commonly affected.

A-type botulinum toxin blocks presynaptic production of acetylcholine, thereby disrupting neuromuscular transmission, and leading to flaccid muscle paresis. Injection of toxin into the internal anal sphincter is a new, minimally invasive, effective method of treating children with hypertonicity / achalasia of the sphincter, pelvic muscle dyssynergia, stool retention, secondary fecal incontinence from rectal overflow. The effect of A-type botulinum toxin on smooth muscles of hollow organs has not been yet studied.

Objective. To analyze the effect of A-type botulinum toxin on the tone of the smooth muscles of the colon in the experiment.

Material and methods. The scientists examined 15 laboratory Sprague Dawley rats. The effect of A-type botulinum toxin on the tone of the smooth muscles of the large intestine was assessed by measuring the stress of the intestinal wall and intraluminal pressure.

Results. The scientists revealed a decrease in pressure in the rectum on the 10th day after the injection of A-type botulinum toxin into the segment of the descending colon. The indices of the intestinal wall tension did not differ before the administration of A-type botulinum toxin, as well as in 10 and 20 minutes. The tension in the administration area of A-type botulinum toxin decreased by 81% on the 10th day after injection. There was an indirect decrease in smooth muscle tone in the segments of the colon above and below the injection by 49 and 80%, respectively.

Conclusion. The decrease in rectal pressure on the 10th day after the administration of A-type botulinum toxin directly depends upon the decrease in the biovoltage of the overlying sections. Further basic research is needed to assess the effect of the toxin on non-targeted smooth muscle.

The article is devoted to the difficulties of differential diagnosis of the etiology of multiple ecchymosis in a newborn child due to the peculiarities of the hemostasis system and a predisposition to bleeding, manifested by extravasation of blood into the skin, mucous membranes and the key role of anamnesis in the final diagnosis.

Objective: to familiarize doctors with one of the rare variants of primary immunodeficiency – chronic granulomatous disease. The authors present a case of late diagnosis of chronic granulomatous disease in a 10-year-old child. The doctors missed the development of complications after BCG vaccination in the form of axillary lymphadenitis on the left (BCGitis), which led to the development of disseminated BCG infection, regarded as an infectious tuberculous process. The onset of chronic granulomatous disease occurred at the age of 10 months (paraproctitis complicated by an anal fistula). The assumption of the presence of immunodeficiency arose in connection with the recurrent course of pneumonia at the age of 4-5 years, but was regarded as a manifestation of a tuberculous infectious process. At the age of 8, during the protracted course of nonspecific polysegmental pneumonia, primary immunodeficiency without specific clinical form was diagnosed on the basis of the results of a comprehensive immunological examination. At the age of 10, chronic granulomatous disease was verified using a study of spontaneous and stimulated chemiluminescence of neutrophils and the results of a genetic study.

The authors describe a clinical case of a protracted course of a new coronavirus infection in a child with the development of severe complications against the background of a hereditary disease of neurocutaneous melanoblastosis, Bloch–Sulzberger syndrome. The progression and severity of the disease was caused by acute onset, lack of specific therapy at the onset of the disease and late seeking for medical help. For 2 months, the patient had long-term persistent SARS-CoV-2 viremia with the development of viral-bacterial pneumonia. A defect in the immune system in the early period of the disease contributed to the protracted course of the disease and the development of complications. The outcome of the disease in this patient was predetermined by the combination of etiological factors tropic to the respiratory system, the presence of background genetic pathology with the development of a pathological response of the innate and adaptive immune system of the body to massive replication of SARS-CoV-2 with the development of the late phase of COVID-19.

Premature birth remains an urgent problem of modern healthcare around the world. At the same time, the improvement of neonatal care for premature babies has led to a significant decrease in infant mortality and an increase in the survival rate of children with very low and extremely low body weight. Nursing and monitoring such children requires a special approach. A premature baby has one or more reasons for the early development of chronic pathology and needs a long-term, differentiated, continuous monitoring, provided a clear continuity of all stages of nursing.

The article presents the results of observation of premature babies in 20 polyclinics of Saint Petersburg in the first year of life at the time of the study. The authors analyzed medical histories (form 112/y) of 88 children born prematurely (from 22 to 36 weeks of gestation). It has been determined that most often the dispensary observation groups include children with pathology of the central nervous system, organs of vision, and surgical pathology. Insufficient coverage with vaccine prophylaxis (from 33 to 78% for various vaccine-preventable infections) was revealed not only with additional drugs for the risk group, but also with the main vaccinations of the National Calendar. Monitoring premature babies, their rehabilitation and habilitation are the responsibility of the third (outpatient) level of nursing. However, the lack of uniform approaches to dispensary observation, rehabilitation treatment of premature babies, the lack of clear routing create difficulties in the management of such children in a polyclinic. The authors proposed options for solving this important problem in order to increase the efficiency of the primary pediatric care for premature babies at the place of residence of the child.

Objective. To evaluate the experience of using a probiotic strain of enterococcus in long-term nutritional support of premature infants.

Characteristics of children and research methods. The children were selected in 2014–2015. The study included 11 premature babies of the first year of life, treated with antibiotics for a long time. Inclusion criteria: prematurity of any severity, signed informed consent from parents to conduct an outpatient rehabilitation program for children, including long-term nutritional support with a probiotic strain of enterococcus. Exclusion criteria: gross congenital malformations; hereditary metabolic diseases, severe forms of organic pathology of the central nervous system. Exclusion criteria: severe forms of somatic and infectious pathology, use of other probiotics. The probiotic strain of enterococcus – E. faecium L3 (No. RU. 77.99.26.009.Е.002272.02.11) in the liquid form (biomass grown on a dairy-free basis) with a titer of at least 10⁸ CFU/ml was orally administered to children 1 ml 2 times a day while eating. The authors evaluated safety and clinical efficacy of long-term use of the probiotic strain of enterococcus.

Results. The authors noted the effectiveness of long-term use of a probiotic strain of enterococcus as nutritional support for premature infants for adequate psychomotor and physical development. They revealed the disappearance of the manifestations of atopic dermatitis while taking a probiotic strain of enterococcus. In the course of the study, the safety of long-term intake of a probiotic strain of enterococcus was established as an integral part of the rehabilitation program for premature babies, who completed their nursing in a hospital.

Conclusion. The continuation of studies to assess the clinical efficacy of long-term intake of probiotics in children of the first year of life who were born prematurely and received antibiotics for a long time can be considered very promising.

Objective. To optimize therapeutic approaches to the treatment of abdominal pain syndrome in children with overlapping syndrome of biliary dyskinesia + irritable bowel syndrome.

Characteristics of children and research methods. The study included 309 children aged from 13 to 17 years. Irritable bowel syndrome was diagnosed in 76 (24.6%) patients in accordance with the Rome IV revision criteria and the results of laboratory and instrumental studies. 45 (59.2%) patients had a variant of the syndrome with constipation, 19 (25%) – a variant with diarrhea, 12 (15.8%) – a mixed variant (alternating constipation with diarrhea). Biliary pain was detected in 58 (76.3%) patients, including 34 (44.7%) patients with constipation, 13 (17.1%) with diarrhea variants and 11 (14.5%) with mixed option. The researchers used two treatment options: trimebutin (Trimedat, ValentaPharm, Russia) in an age dosage for 28 days (main group) and papaverine in an age dosage for 4 weeks (comparison group).

Results. The main group demonstrated a more pronounced decrease in the intensity of abdominal pain associated with both irritable bowel syndrome and biliary dyskinesia. Differences between treatment results in the main and control groups are statistically significant (p<0.01). During the treatment period there were no cases of side effects in the main group, the differences in the frequency of side effects between the main group and the comparison group are statistically significant. (p=0.01).

Conclusion. Correction of abdominal pain with trimebutin significantly exceeds the effectiveness of standard antispasmodic therapy in the treatment of overlap syndrome of irritable bowel syndrome + biliary dyskinesia. Trimebutin therapy is safe and can be recommended as a fairly effective method of treating children with overlap syndrome.

Rhinitis is characterized by nonspecific symptoms, but they reduce the quality of life as much as possible. Within the framework of this work, the clinical efficacy of drugs based on tuaminoheptane and xylometazoline was evaluated. To achieve this goal, the authors initiated a prospective single-center non-randomized comparative pilot study, the task of which was to compare the effectiveness of intranasal drugs containing tuaminoheptane sulfate and acetylcysteine (Rinofluimucil) and xylometazoline + seawater in pediatric patients with acute uncomplicated rhinitis.

Characteristics of children and research methods. The study included patients who met the inclusion / exclusion criteria, they were divided into 2 groups after a standard ENT examination (patients of Group 1 were prescribed with tuaminoheptane sulfate and acetylcysteine (Rinofluimucil), patients of Group 2 were prescribed with a combination of xylometazoline and sea water. The study included 61 patients (32 boys and 19 girls aged 4 to 16 years): 30 patients in Group 1 (tuaminoheptane sulfate and acetylcysteine, Rinofluimucil) and 31 patients in Group 2 (xylometazoline + sea water); comparable by sex and age.

Results. Comparison of the patients’ condition at the beginning and at the end of the treatment course demonstrated that both drugs had a therapeutic effect: for each of the assessed indicators in both study groups, there was a pronounced positive trend (statistically significant for all studied symptoms, p<0.01). None of the observed patients during treatment demonstrated unwanted side effects and complications in the middle ear.

Conclusion. Intranasal use of a combination of tuaminoheptane sulfate and acylcysteine (Rinofluimucil) has shown efficacy comparable to that of an intranasal spray containing xylometazoline in the treatment of acute uncomplicated rhinitis in children. The advantage of a drug based on tuaminoheptane is the expected gentle effect on the mucous membrane of the nasal cavity and the additional therapeutic effect of the original acetylcysteine, which is characteristic of Rinofluimucil.

Hemolytic uremic syndrome associated with Streptococcus pneumoniae is a rare disease that occurs in 5-10% of all cases of hemolytic uremic syndromes in childhood. Pneumonia and meningitis are among the main diseases that can be complicated by St. pneumoniae-associated hemolytic uremic syndrome. The widespread introduction of polymerase chain reaction into clinical practice has increased the frequency of detection of this pathology. The article presents the epidemiology, clinical picture, features of pathogenesis, diagnosis and treatment of St. pneumoniae-associated hemolytic uremic syndrome. The authors described 3 clinical cases of this syndrome in children with pneumonia. In 2 patients, pneumonia proceeded with destruction of lung tissue, pleural empyema, bacteremia, and severe renal failure, which required hemofiltration therapy to replace renal function. Outcomes of hemolytic uremic syndrome associated with St. pneumoniae is worse than in typical hemolytic uremic syndrome, and is associated with the severity of the underlying disease (pneumonia, meningitis).

The inclusion of specialized food products intended for a specific category of the population is one of the measures aimed at improving the nutritional structure of children. The baby food industry is constantly evolving to meet the time challenges. New directions include the production of dishes of a high degree of readiness for baby nutrition. The direction of the production of ready-made meals has not yet been formed in baby nutrition and has broad prospects. The use of modern heat treatment technologies, including the production of dishes with a short shelf life, allows you to bring products and dishes as close as possible to homemade ones.