An interview with Professor Mazankova Lyudmila Nikolayevna, Dr. Med. Sci., Head of Chair of Children’s Infectious Diseases at the Russian Medical Academy of Continuous Professional Education of the Ministry of Health of Russia, Chief External Specialist in Children's Infections in the Central Federal District of the Ministry of Health of Russian and the Moscow Healthcare Department. An Honoured Doctor of the Russian Federation.

Lyudmila Nikolayevna told us about her path in the profession: the impact of one clinical case on the area of her scientific activities; about new guidelines for the preventive vaccination against pneumococcal disease in children and adults; about the vaccines, which will appear in the national calendar in the near future; and about the fight against anti-vaccination lobbyists.

Also, the interview addressed such important topics as the rising number of COVID-19 cases in children, prevailing strains and possible measures to prevent infectious diseases.

Aim. To analyze current data on epidemiology, pathophysiology of eosinophilic gastrointestinal diseases (EGID) in children with focus on small intestinal affection.

Key points. EGID are inflammatory conditions of the gastrointestinal tract, characterized by tissue eosinophilia with a depth of organ damage from the mucous layer to the muscular and serous layer, resulting in severe damage to their structure and function. Importance of food allergy is still underestimated, although it affects about 5% of children and up to 3–4% of adults. Eosinophilic diseases of the digestive tract are receiving close attention from experts all over the world. A few decades ago, these diseases were described as rare and articles presented isolated clinical cases, but nowadays the publications have increased by several times. There is increasing evidence of the role of aeroallergens and food allergens in the pathogenesis of eosinophilic esophagitis, gastritis, and enteritis, as children and adults with EGID are often found to have positive skin tests for food allergens and/or a family history of allergic diseases.

Aim. Based on literature data, to analyze the clinical significance of the milk fat globule membrane in the nutrition of newborns and young children.

Key points. Breast milk has been and remains the “gold standard” for feeding a child of the first year. The composition of modern formulas is based on the new information on the breast milk components, which have the most important programming impact on the growing child body. Currently, scientists pay special attention to the optimisation of the fat component of formulas with the introduction of milk fat globule membranes — a unique structure surrounding glycerine nucleus and comprising complex lipids in the form of phospholipids, gangliosides and cholesterol.

Conclusion. The scientific studies demonstrated the critical role of “minor” lipids in the process of postnatal formation of the central nervous system and visual analyzer, the formation of the immune system and the regulation of metabolic processes. It is now reliably known that formulas with milk fat globule membranes are well-tolerated and have favourable effect on the prevention of infectious diseases and lipid exchange status.

Aim. To assess the effectiveness of skin cosmetics in skin restoration (decrease in dryness, irritation, itching) in children up to 7 years

Design. An observative open clinical noncomparative study.

Materials and methods. The study enrolled 50 children: 30 children with sensitive skin and 20 participants with head cradle caps. The study included 2 visits in the office. During the examination, the doctor assessed the severity of dryness and itching in children with sensitive skin, and the severity of dryness, itching and flaking of the scalp in children with cradle cap. The values varied 0 to 10 points. The duration of the study was 28 ± 3 days.

Results. Within usage of the cosmetics, there was no negative effect on the skin. No patients require external anti-inflammatory treatments. Within washing, there was no lacrimation, conjunctiva irritation. Day 3–5 decreased dryness of the sensitive skin approximately twice, light itching was present only in 1 participant. At the end of the study, minimal skin dryness was observed in only 2 patients. On Day 3–5 of shampoo usage, the cradle cap group has had no complaints of itching and head skin dryness has significantly reduced. At the end of the study, the dryness, itching and squamous elements median values in all the patients was significantly lower.

Conclusion. Regarding skin peculiarities in children up to 7 years old, it is quite important to find appropriate variants (ingredients and formulas) of skin management because without risk factors and taking into account hygiene rules increased sensitivity to the environment may be decreased leading to lower risk of irritation and skin conditions. Cosmetic components might positively influence skin moistening and maintain optimal barrier function.

Aim. To analyze the obesity prevalence in children and adolescents of the Novosibirsk region for the period 2018–2022, to assess the state of lipid and carbohydrate metabolism in children with primary obesity of 1–2 degrees without arterial hypertension.

Design. An observational, descriptive, cross-sectional study.

Materials and methods. The analysis of statistical indicators according to the annual reporting forms of federal statistical observation No. 12 in children and adolescents of the Novosibirsk Region and the patient database of the City Center for Endocrine and Metabolic Disorders in the Children's City Clinical Hospital No. 1 of the Novosibirsk Region for 2018–2022 was carried out. We also examined 154 children aged 6–17 years with primary obesity of 1–2 degrees, without arterial hypertension in medical history and at the time of examination; their standard lipid profile, fasting blood glucose, insulin, oral glucose tolerance test, Homeostasis Model Assessment (HOMA) and Triglyceride[1]Glucose Index (TyG) were examined.

Results. According to the data of report form No. 12 in the Novosibirsk Region for 2018–2022, obesity is the leader among the endocrine system diseases in children and adolescents. The maximum number of cases of obesity among children and adolescents was registered in 2019 (9030 people; 1560.7 cases per 100 thousand population), the minimum — in 2021 (7289 people; 1235.6 cases per 100 thousand population). Among the examined children with primary obesity of 1–2 degrees, impaired glucose tolerance was detected in 5.2%, changes in the standard lipid profile — in 33.8%. The group of children with insulin resistance estimated by the HOMA index above 3.16 was 46 people (29.9%), according to the TyG index — above 7.98 — 78 children (50.6%).

Conclusions. According to statistical reporting, the maximum number of cases of obesity among children and adolescents was registered in 2019, the minimum — in 2021, the incidence rate is lower than the prevalence of pathology in Russia. The highest prevalence of obesity was noted in the age group of 15–17 years. The revealed high comorbidity for the main components of the metabolic syndrome in patients with 1–2 degrees of obesity indicates the need for early examination, correction and monitoring of key cardiometabolic risk factors in this patients group.

Aim. To present modern views on various types of eating behavior in children and adolescents and their role in the development of obesity.

Key points. Both hereditary predisposition factors and environmental factors play a role in the formation of obesity, among which eating behavior (PP) plays a significant role. Various questionnaires and scales are used to study PP disorders, one of the validated questionnaires is the Dutch Eating Behavior Questionnaire (DEBQ), which plays an important role in clinical practice, it allows you to simultaneously evaluate three main types of PP, which is important in the diagnosis of early PP disorders and contributes to the correction of identified disorders, as well as it allows to increase the effectiveness of therapeutic and preventive measures.

It is also an urgent issue, especially given the limited number of medications allowed for the treatment of obesity in childhood and adolescence, is the search for approaches that can effectively treat obesity, especially in the long term, and therefore it is necessary to rely, inter alia, on knowledge of the peculiarities of eating behavior in patients in order to optimize the trajectory of treatment.

Conclusion. In all cases, treatment of PP disorders should include a full, in-depth, detailed analysis of PP to understand the underlying factors and stress. Interventions should be tailored to each case, with the goal of restoring normal nutrition and nutritional intake and improving the patient's quality of life.

Aim. Аnalysis and synthesis of the literature data on the problem of differential diagnosis of neonatal progeroid syndrome.

Key points. One of the rarest representatives of premature aging syndromes is neonatal progeroid syndrome (Wiedemann–Rautenstrauch syndrome). It is an ultra-orphan disease with autosomal recessive type of inheritance, associated with a mutation in the POLR3A, POLR3B, POLR3GL genes and characterized by congenital lipodystrophy and premature aging.

The disease manifests from the first days of life: low body length and weight at birth, pronounced phenotypic features (pseudohydrocephaly, progeroid facial features, generalized lipodystrophy, neonatal incisors). Severe bronchopulmonary and skeletal damage is seen over the course of life, and average life expectancy ranges from 7 months to 2 years but can reach 27 years. The differential diagnosis is made with Hutchinson–Gilford syndrome (progeria), which clinical signs manifest at 1.5-2 years of age, and with Marfan-progeroid lipodystrophy, Fontaine syndrome, and Sekkel syndrome.

Conclusion. Early diagnostics is necessary for predicting the course of the disease, selection of treatment, and determining of further management.

Aim. To identify the features of clinical and anamnestic indicators in children of the first year of life with impaired motor development, born at different stages of gestation.

Design. Сomparative study.

Materials and methods. 64 children of the first year of life with motor development disorders were examined. 4 groups were formed: 1st (n = 16) — full-term, 2nd (n = 16) — late preterm, 3rd (n = 16) — moderately premature, 4th (n = 16) — extremely preterm children. All children underwent an examination, which included an assessment of psychomotor development using the L.P. Zhurba and E.M. Mastyukova scale and studying the features of the course of their anamnesis.

Results. Very premature children had a greater severity of cerebral ischemic and respiratory disorders in the neonatal period compared to full-term, late preterm and moderately premature patients (p < 0.001). Total score of psychomotor development according to the L.P. Zhurba and E.M. Mastyukova scale in patients of all study groups was below standard values (p < 0.001), while the total score in very premature infants was lower than in full-term infants (p = 0.004).

Conclusion. The results obtained can serve as the basis for the development of a personalized approach to planning treatment and rehabilitation activities for children with motor development disorders.

Introduction. Epilepsy has a different nature and is characterized by a variety of clinical manifestations. The combination of epilepsy and movement disorders raises one of the most serious questions in neurorehabilitology: is the rehabilitation effect itself possible in epilepsy? At present, this issue cannot be considered resolved due to ongoing discussions.

Aim. To determine the long-term results of the rehabilitation of young children suffering from epilepsy accompanied by motor dysfunction.

Design. Single-center retrospective study.

Materials and methods. Тhe study group consisted of 38 patients (19 girls and the same number of boys) with epilepsy and movement disorders, who underwent rehabilitation treatment from 2005 to 2015 in the following volume: in the 1st group — medical massage, in the 2nd — Vojta-therapy and in the 3rd — a combination of these methods. The cycle consisted of three courses of treatment with an interval of 2 to 5 months. The course included 10–15 sessions for 25–30 minutes daily. A follow-up assessment of the state of motor functions was carried out in the period from 2020 to 2021, the average follow-up period was 121.89 ± 5.32 months, Me = 126.00 [96.00; 156.00], SD 32.84 after the end of medical rehabilitation during this period, patients continued to receive rehabilitation measures.

Results. Upon completion of the cycle of rehabilitation measures, a positive result was achieved in all 3 groups. We compared the indicators obtained at the end of rehabilitation and according to follow-up data. The most pronounced was the positive difference between the indicators after the end of the course and those obtained in the follow-up in the 3rd group (χ2 Pearson = 6.533; p = 0.038). In the 2nd group, the difference was also statistically significant (χ2 = 27.137; p = 0.001), but less than in the 3rd group. In the 1st group, the differences were the smallest, but, nevertheless, statistically significant (χ2 = 39.000, p = 0.0001). Thus, in all groups, a progressive improvement on the GMFCS scale was demonstrated, while in the 3rd group the result was the best.

Conclusion. Thus, the improvement on the GMFCS scale achieved during the rehabilitation of patients with epilepsy and impaired motor function can not only be maintained, but also improved.

Aim. To systematize modern scientific research that studies the role of monocytic chemoattractant in the development and progression of various diseases.
Key points. At present, the interest of the scientific community in the issues of the pathogenetic role of the monocyte-macrophage link of the cytokine spectrum in the development of a number of pathological changes in the body has increased.
Conclusion. The monocyte chemoattractant is one of the key components of endothelial dysfunction and possibly can be presented as a biological marker of various diseases, which will greatly assist in early diagnosis and organization of the treatment process accordingly.

Aim. To demonstrate a clinical case of autoimmune hepatitis type 1 in a patient aged 10 years.

Key points. Certain infections can cause frequently asymptomatic and self-limited hepatitis, while others may result to severe acute hepatitis and/or act as a trigger for autoimmune hepatitis in young children. We present the case of a previously healthy 10-year-old girl with a syndrome of cytolysis and cholestasis who had elevated serum transaminases, Epstein — Barr virus (EBV) serology compatible with recent EBV infection, and positive anti-smooth muscle antibody characteristic of onset autoimmune hepatitis type 1. Liver biopsy was also typical of autoimmune hepatitis as attested by the presence of portal inflammation and interface hepatitis. Persistent EBV-related hepatitis was excluded by negative serum EBV-PCR.

Conclusion. The presented clinical case suggests that in children with cholestatic hepatitis, positive EBV serology cannot exclude the presence of other causes of liver disease. In this context, autoimmune hepatitis should be considered as an alternate diagnosis, particularly when there is specific liver-related autoantibody detection. In such conditions, a liver biopsy seems mandatory to achieve a correct and on-time diagnosis of a poor prognostic disease such as autoimmune hepatitis.

Aim. To update the information about hereditary agammoglobulinemia, to describe a clinical case.

Key Points. The frequency of hereditary agammaglobulinemia varies from 1:100,000 to 1:200,000. The cause of this disease is a gene mutation on the long arm of the X chromosome that codes for B-cell tyrosine kinase. The disease is characterized by a sharp decrease in all classes of serum immunoglobulins and the number of circulating B - lymphocytes. Hereditary agammaglobulinemia manifested by repeated bacterial infections during the first two years of life. Patients require lifelong replacement therapy with intravenous immunoglobulins.

In this article we included 3 own clinical observations.

Conclusion. The quality of life and prognosis of the patients with Bruton's disease depends on the early detection of the disease and the quality of medical care provided