Background. The approaches to the managing of nutrition for healthy children are not always applicable to patients with neurological disorders, since their body composition, muscle tone, level of physical activity, and energy requirements significantly differ from healthy children and, therefore, must be personalized with the mandatory involvement of a nutritionist/dietitian.

The purpose is to assess nutrition in children with cerebral palsy (CP) depending on Gross Motor Function Classification System (GMFCS) level.

Materials and methods. Eighty-eight 2 to 16 years nine months CP children rehabilitated at the National Medical Research Center for Children’s Health from 2019 to 2021 were included in this study. Participants were divided into two groups. The main group was patients with GMFCS levels IV–V (48 children) and the comparison group — GMFCS levels I–III (40 children). The medical and dietary history of participants was analyzed. Anthropometric parameters using the WHO AnthroPlus (2009) software were assessed in all participants, the ability to eat and drink was determined by questioning the EDACS scale (Eating and Drinking Ability Classification System).

Results. The mean values of weight/age, height/age, BMI/age Z-scores were significantly below 0 (p = 0.01) in all patients (88): WAZ –1.54 ± 1.9, HAZ –1.03 ± 1.48, BAZ –1.5 [–3.11; –0.35]. Children of the main group had significantly lower Z-scores. As a result of the survey, patients of the main group were revealed to have indicators characterizing eating disorders significantly more often (p < 0.001) than the comparison group. EDACS survey revealed that children of the main group were significantly more likely (58% and 10%, respectively; p < 0.001) to have disorders corresponding to levels IV–V, characterized by significant safety restrictions. We have developed a nutritional assessment and management algorithm for children with cerebral palsy, admitted for rehabilitation at the neurological department.

Conclusion. Children with cerebral palsy need regular nutritional assessment and management for timely and adequate nutritional support, including enteral nutrition.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: December 28, 2021
Accepted: February 17, 2022
Published: March 15, 2022

Introduction. Malabsorption syndrome is one of the most common manifestations of intestinal insufficiency in cystic fibrosis (CF). CF being the cause of protein-energy malnutrition, so diet therapy using specialized enteral nutrition products is an integral part of CF complex treatment.

Purpose. Evaluation of the tolerability and effectiveness of the domestic specialized semi-elemental formula based on hydrolyzed milk protein «NUTRIEN® Elemental» in the treatment of protein-energy malnutrition in CF children older than 1 year of age with malab-sorption syndrome.

Materials and methods. Under supervision were 32 CF one year 3 months to 17-year children with a mixed form of the disease and malnutrition. Twelve (38%) children had hypolactasia. Nutritional status was assessed using the WHO-Anthro for 1–5-year children  and WHO-Anthro Plus for patients over 5 years of age) programs, and indices of clinical and biochemical blood tests. The average du-ration of follow-up was 21 day. Statistical processing was performed using «Statistica 6.0» («StatSoft Inc.», USA) and Microsoft Excel 2010.

Results. While taking a specialized product, there was a decrease in the number of patients with severe and moderate forms of protein-energy malnutrition, a significant upward trend in such biochemical markers of nutritional status as prealbumin (p < 0.01), transferrin (p < 0.01), total protein (p < 0.01).

Conclusion. The use of a domestic specialized product based on hydrolyzed milk protein «NUTRIEN® Elemental» in the diet therapy of sick children with malabsorption syndrome, including CF patients, indicates its positive effect on the indices of the nutritional status of patients and the course of the underlying disease.

Conflict of interest. The authors declare no conflict of interest.

Acknowledgement. The study had no sponsorship.

Received: January 27, 2022
Accepted:  February 17, 2022
Published: March 15, 2022

Goal. To evaluate the safety profile of onasemnogene abeparvovec (Zolgensma®) gene therapy in children with spinal muscular atrophy (SMA) in real clinical practice.

Materials and methods. The study included 30 SMA children who underwent gene replacement therapy with onasemnogene abeparvovec (Zolgensma®) from December 2020 to December 2021 at the Center for Pediatric Psychoneurology. All children had a diagnosis of SMA confirmed by molecular genetic methods, with no more than 3 copies of the SMN2 gene and the absence of antibodies to the adeno-associated virus serotype 9. The safety profile was assessed by monitoring the clinical and laboratory data of the patients after administration of onasemnogene abeparvovec. Clinical events included all changes in the child’s condition that could be associated with the administration of the drug (hyperthermia, decreased appetite, nausea, vomiting, stool disorders). Laboratory assessment included monitoring of complete blood count, biochemical blood tests, blood coagulation indices. The degree of laboratory changes was estimated according to common terminology criteria for adverse events (CTCAE version 5.0).

Results. The safety profile of the drug Zolgensma® was studied in 30 children aged from 3 to 39 months, with a weight of 5.2 kg to 14.2 kg.

Twenty-eight (93.3%) children had at least one clinical event associated with the administration of the drug. Hyperthermia was observed in 24 (80%) children, nausea and vomiting in 18 (60%) children, decreased appetite in 20 (66.7%) children, stool changes in 4 (13.3%) children. Monitoring of laboratory indices revealed thrombocytopenia and monocytosis in twenty-two (73.3%) children and neutropenia in twelve (40%) children in the general blood test. An increase in the level of transaminases was noted in all children, the CTCAE grade 1 was detected in fifteen (50%) children, the CTCAE grade 2 in 7 (23.3%) children, CTCAE grade 3 in 6 (20%) children, CTCAE grade 4 in 2 (6.7%) children. Children with CTCAE grade 3 required correction of the prednisone dose up 2 mg/kg per day for 2-4 weeks. Two children with CTCAE grade 4 required pulse therapy with methylprednisolone at a dose of 30 mg/kg per day. Regardless of the level of transaminases, no change in the level of total and direct bilirubin was observed in any case. A decrease in prothrombin time was observed in 14 (46.6%) children. An increase in the level of troponin I was detected in four (13.3%) children. In all cases of serious adverse events, according to laboratory indices, the clinical condition of the children remained stable. The average duration of prednisone intake was 17.8 ± 6.6 weeks.

Conclusion. The safety profile of the drug onasemnogene abeparvovec (Zolgensma®) in real clinical practice in children with SMA is presented.

Contributions:
Kuzenkova L.M., Uvakina E.V., Popovich S.G., Podkletnova T.V. — concept and design of the research;
Nezhelskaya A.A., Abdullaeva L.M., Fisenko D.A., Naidenko A.V. — collection and processing of materials;
Kuzenkova L.M., Uvakina E.V., Popovich S.G., Podkletnova T.V. — text writing;
Kuzenkova L.M. — editing.
Аll co-authors — аpproval of the final version of the article, responsibility for the integrity of all its parts.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 10, 2022
Accepted: February 17, 2022
Published: March 15, 2022

Introduction. Currently, worldwide the formation of cerebral palsy (CP) makes a great contribution to the gain in childhood disability prevalence. The goal is to conduct a comparative analysis of risk factors and the main causes of the development of CP in children living in the Belgorod region for the period from 2020 to 2021.

Materials and methods. There were analyzed histories of two hundred two CP children aged from four months to seven years.

Results. Spastic forms of the disease predominated in the structure of infantile CP. Significantly more often this pathology occurred in premature babies at an early age and children residing in urban areas. The most frequent risk factors for the onset of the disease were such pathology of pregnancy as chronic fetoplacental insufficiency and chronic intrauterine fetal hypoxia. Delayed psycho-speech development had a leading place among all manifestations of the central nervous system in CP patients is beyond doubt. An analysis of the causes leading to the onset of the disease showed that in most cases it is not possible to discriminate one of them, since a combination of several unfavourable factors is often noted during pregnancy and parturition. CP children require constant care from relatives, long courses of complex drug and rehabilitation treatment.

Contribution:
Kryuchkova T.A., Balakiryeva H.A., Matviyenko H.V. — concept and design of the study;
Balakiryeva Е.A., Matviyenko Е.V. — collection and handling of materials;
Kryuchkova T.A. — analysis of data obtained, writing up the ma­nuscript.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 08, 2022
Accepted:  February 17, 2022
Published: March 15, 2022

The aim of the work is to form a mathematical model of viral hepatitis based on structural modelling, discriminant and factorial analysis of laboratory parameters of patients.

Materials and methods. The data array included the results of a comprehensive laboratory testing of 109 children with viral hepatitis B and C (33 parameters).

Results. Seven main factors in the pathogenesis of viral hepatitis in children reflect the severity of endogenous intoxication and disorders of protein metabolism, modification of the mode of functioning of enzymes, and supramolecular multi-enzyme complexes in conditions of progression of the first two components.

Conclusion. The identified factors of the pathogenesis of viral hepatitis may reflect the endogenous intoxication during disease progression, the state of detoxification enzyme systems, and the further fibrotic changes in the liver.

Contribution:
Martusevich A.K. — the concept and design of the study, the collection and processing of material, writing the text;
Galova E.A. — providing material for research;
Korkotashvili L.V. — carrying out mathematical modeling;
Popovicheva A.N. — editing the text of the article.
Аll co-authors — аpproval of the final version of the article, responsibility for the integrity of all parts of the article.

Informed consent: informed consent was received from the patients parents for the publication of a description of the clinical case.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: January 14, 2022
Accepted: February 17, 2022
Published: March 15, 2022

Introduction. Cystic fibrosis (CF) is a hereditary, multisystem disease that is accompanied by numerous complications, which affects the quality of life (QOL) of patients, determining its duration and comfort. With an increase in the survival rate of CF patients, various concomitant diseases were identified, one of which is cystic fibrosis-associated (dependent) diabetes mellitus (CFDM). Traditionally, it is believed that CFDM worsens the patient’s QOL. To establish this, a questionnaire is conducted with validated questionnaires for children — Health Utilities Index (HUI).

Objective: to determine the QOL in CF patients with disorders of carbohydrate metabolism.

Materials and methods. There were surveyed sixty-four CF 5 to 18 year patients (Me = 13.59 years), including 42 girls and 22 boys. To diagnose disorders of carbohydrate metabolism, a standard oral glucose tolerance test (OGTT) was performed in all patients. According to OGTT data CF patients were divided into 3 groups: 1^st — 25 cases without deteriorations of carbohydrate metabolism; 2^nd — 25 children with prediabetes and 3^rd — 14 CFDM patients on insulin therapy and without it. The assessment of the psychological state and development of CF patients with prediabetes and CFDM cases was carried out. The parameters of emotional well-being, attitude to the disease and adherence to treatment were studied.

Results. The authors revealed no significant difference in the QOL in CF patients by all scales of the questionnaire. There was a tendency to decrease in QOL according to the overall score of the questionnaire (HUI3) in CF children with prediabetes (0.77 ± 0.18 units) compared with patients without carbohydrate metabolism disorders and CFDM patients (0.81 ± 0.14 units and 0.80 ± 0.14 units, respectively). In the emotional sphere, there is a tendency to decrease in QOL in patients with prediabetes and CFDM cases. The analysis of indices on separate scales revealed a more pronounced decrease in QOL on the emotion scale in CF patients with disorders of carbohydrate metabolism. CFDM patients are in a more severe psychological state than children with prediabetes. They are significantly more likely to experience psychophysical discomfort and tend to fixate on the symptoms of the disease (Fi­sher’s angular transformation criterion 3.11, p < 0.01). These patterns should be taken into account when analyzing the psycho-emotional state of a CF child for timely prevention and correction.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 14, 2022
Accepted: February 17, 2022
Published: March 15, 2022

Introduction. An essential point in the prevention and risk assessment of these diseases is identifying their predictors predisposing to a progressive course of inflammation in the gastroduodenal zone, particularly gastroesophageal reflux disease (GERD) intersection syndrome and dyspepsia syndrome (DS). A more unfavourable situation is observed among the indigenous — Mongoloid populations.

The aim of the study is to investigate the ethnic features of the prevalence and clinical manifestations of the intersection of GERD with DS in schoolchildren of Mongoloid and Caucasian populations of Tuva, Evenkia and Buryatia.

Material and methods. A cross-sectional survey was carried out on the presence of gastroenterological complaints in schoolchildren aged 7–17 years, followed by random gastroscopy with a biopsy of the gastric mucosa. Six hundred forty one Caucasians, 894 Mongoloids were examined in the Republic of Tuva; in Buryatia, there are 168 Caucasians, 622 Mongoloids; in Evenkia, there are 993 Caucasians, 376 Mongoloids. Endoscopically examined: in Tuva, 122 Caucasians, 158 Mongoloids; in Buryatia — 28 Caucasians, 82 Mongoloids; in Evenkia, there are 133 Caucasians, 71 Mongoloid.

Results. Among schoolchildren in the Siberian region, the prevalence of GERD overlap syndrome with DS was 4.7%. An increase in indices was noted among the Mongoloid populations: by 5.7% and 3.6% among Caucasians. Schoolchildren of Mongoloid populations with the overlapping syndrome often had a variant of dyspepsia with epigastric pain. At the same time, regardless of ethnicity, schoolchildren with intersection syndrome have an increased likelihood of forming erosive and ulcerative lesions of the mucous membrane of the gastroduodenal zone.

Conclusion. The syndrome of overlapping in Siberian schoolchildren has a more unfavourable course with features in the ethnic populations of Mongoloids and Caucasians.

Contribution:
Polivanova T.V., Vshivkov V.A. — concept and design of the study;
Kasparov E.V., Vshivkov V.A. — collection and processing of the material;
Polivanova T.V. — writing the text;
Vshivkov V.A. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all its parts.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: November 19, 2021
Accepted: February 17, 2021
Published: March 15, 2021

The review shows аtopic dermatitis (AD) as a chronic inflammatory skin disease that develops in early childhood in infants with a hereditary predisposition. The inflammatory response type 2, including a complex interaction of type 2 T-helpers (Th2), congenital lymphoid cells, granulocytes (including eosinophils, mast cells and basophils), cytokines (IL-4, IL-5, IL-13 etc.) and immunoglobulin E (IgE) underlie at the base of the AD pathogenesis. IL-4 and IL-13 deserve special attention since they affect several links of pathogenesis at once. Both cytokines interact with receptors. Their critical subunit is the alpha chain of the IL-4 receptor. This molecule is the target for dupilumab, the first genetically engineered biologically active drug that demonstrated the high efficacy in the treatment of AD in 6 to 18-year children patients. At the same time, the data available in the literature indicates the drug to have both nonspecific (soreness at the injection site, allergic response) and specific (conjunctivitis, secondary herpetic infections, eosinophilia) adverse effects, which should be taken into account by practitioners when prescribing immunobiological therapy.

Conclusion. In randomised, double-blind, placebo-controlled trials in children of different ages with the moderate to severe course of AD, immunobiological therapy with dupilumab has demonstrated high clinical efficacy in the form of a rapid remission of the disease and exemplary safety.

Contribution:
Larkova I.A. — article concept and text writing;
Glukhova E.A. — collection and processing of material;
Revyakina V.A. — article concept, editing.
Аll co-authors — аpproval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: December 24, 2021
Accepted: February 17, 2022
Published: March 15, 2022

The lecture presents modern information about embryogenesis, associated anomalies, diagnosis, and treatment of various forms of anorectal malformations (ARM) in children. During the initial examination of ARM children the authors recommend the following sequence of actions for a neonatologist and a surgeon: catheterization the esophagus in order to exclude combined esophageal atresia; survey radiography of the abdominal organs in direct or lateral projections to exclude concomitant duodenal atresia, dilated intestinal loops and primary assessment of the condition of the spine and sacrum; echocardiography to detect concomitant heart and large vessel defects (occur in 30% of ARM newborns), Ultrasound of the kidneys and bladder. A special X-ray study to determine the location of the intestine al atresia is carried out by a pediatric surgeon no earlier than 18–24 hours after birth in newborns without an anus on the perineum. It is believed that after this period of time, the gas will fill the atresized part of the intestine, which is determined on the side image at the level of the coccyx or sacrum or in the abdominal cavity, depending on the type of pathology. To determine the prognosis of the functional state of the pelvic organs before surgery, it is recommended to determine the sacral index, since a visual assessment of the state of the sacrum and coccyx (counting the number of vertebrae) in most cases does not allow a quantitative assessment of the detected changes. The bowel management program provides training for the patient’s parent or child on how to empty the bowel in order to achieve a “clean” period (without constipation and fecal smearing) during the day. The schedule is developed individually for each patient, usually within one week. The issues of postoperative rehabilitation of patients at the stage of treatment in the clinic are discussed.

Contribution:
Khvorostov I.N. — research concept and design of the study, editing;
Khvorostov I.N., Okulov E.A., Dotsenko A.V. — collection and processing of material, text writing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 04, 2022
Accepted:  February 17, 2022
Published: March 15, 2022

Introduction. To assess the features of the course of new coronavirus infection over the first month of life.

Materials and methods. The article presents four clinical cases of coronavirus infection in newborn children aged 8 to 30 days, treated at the covid centre of the National Medical Research Center for Children’s Health.

Results. The incubation period lasted from 2 to 10 days. The clinical picture included prevalence of hyperemic palatine arches, lethargy, impaired sucking, hyperemia of the conjunctiva, sneezing, mucous discharge from the nose, cough of varying severity. In more severe cases, an elevation in body temperature, a change in the auscultatory picture of lungs, abnormalities in the electrocardiogram. In the general blood test, there was noted anaemia, relative and absolute neutropenia, lymphocytosis and monocytosis; enlargement of аctivated partial thromboplastin time, increasing the level of D-dimer in the coagulogram. Computed tomography of the chest showed focal infiltrative changes in the lung tissue or uneven pneumatisation.

Conclusion. Our observations show coronavirus infection in newborns, most often to show a mild and moderate course, which does not require the appointment of antibacterial drugs and hormonal, and anticoagulant therapy without strict indications. According to the recommendations of the World Health Organization, the mother, and baby should remain together throughout the day, regardless of the suspicion or confirmation of a viral infection. To date, there is no conclusive evidence mother-to-child transmission of COVID-19 through breastfeeding is possible, and the benefits of breastfeeding far outweigh the minimal risk of infection. In addition, IgA and IgG to SARS-CoV-2, detected in milk, neutralise the effect of the virus.

Contribution:
Kharitonova N.A. — concept and design of the study;
Illarionova M.S., Nesterovich M.I., Mitish M.D., Seliverstova A.A. — collection and processing of the material;
Kharitonova N.A., Basargina M.A. — statistical processing;
Kharitonova N.A. — writing the text;
Basargina M.A. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received:  January 20, 2022
Accepted: February 17, 2022
Published: March 15, 2022

Currently, the method of ultrasound diagnostics (echography) is the leading one among other imaging tools in medicine. The history of the method begins in 1942, but the main developments should be attributed to the 1950–1960s, when owing to the first ultrasound machines created in various countries, the fundamental possibility of visualizing tissue structures of various organs in normal and in various forms of pathology was proved. Since the 1970s, the solution of technical problems has led to the creation of ultrasound systems close to modern types of equipment. In the same years, ultrasound examinations for the first time began to be effectively used in the Research Institute of Pediatrics of the USSR Academy of Medical Sciences, where they took a worthy place among other diagnostic methods. For the first time, ultrasound techniques of various organs were developed, their normal age characteristics were established, the possibilities in the diagnosis of many forms of pathology were proved and the criteria for their differential diagnosis were determined. But at the same time, despite the significant diagnostic potential of ultrasound diagnostics, we must not forget about the sufficient subjectivity of the method, which requires special attention and knowledge when obtaining and interpreting echographic images. The newest ultrasound methods that have appeared in recent years include three- and four-dimensional echography, ultrasound tomography, the possibility of panoramic, contrast and intra-organ studies, pulse inversion harmonics, quantitative assessment of organ parenchyma, elastography, various variants of Doppler (including intravascular) methods.

Contribution:
Dvoryakovskiy I.V., Dvoryakovskaya G.M. — concept and design of the study;
Dvoryakovskiy I.V., Trukha­nov A.I., Dvoryakovskaya G.M. — collecting the data, writing and editing the text;
Smirnov I.E. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 14, 2022
Accepted:  February 17, 2022
Published: March 15, 2022