卷 12, 编号 4 (2021)

Background. It is known that the earlier a person’s body weight exceeds the normal range, the metabolic disorders associated with obesity will form at an earlier age. The progressive increase in the prevalence of obesity and metabolic syndrome in different countries is primarily associated with the so-called “human risk factors”, which include: physical inactivity, excessive consumption of food rich in fats and carbohydrates, stress, smoking. In this regard, it is extremely important to regularly monitor the body weight of children and adolescents in order to early identify a tendency to increase body weight for the making recommendations for maintaining weight within the normal range.

Aim. To evaluate the frequency of obesity and overweight in children, adolescents and adults from among the residents of St. Petersburg, to conduct a comparative assessment of the data obtained.

Materials and methods. The work was attended by students of St. Petersburg schools (children and adolescents) and patients (adults) who are being treated in St. Petersburg State Medical Institution “Elizavetinskaya Hospital”. The sample was random: when collecting data from children and adolescents, data from one of the classes in each parallel from 4^th to 11^th grade were taken into account, when collecting data from adults – 2 people from each ward of the gastroenterology department of the St. Petersburg State Medical Institution “Elizavetinskaya Hospital”. Data collection was carried out in the period: August–December 2020. Statistical processing was performed out using the computer software package SPSS 8.0. Estimation of anthropometric parameters (age, body weight, height) and calculation of body mass index (BMI) were performed in 74 children (age 9–12 years), 137 adolescents (age 13–18 years) and 55 adults (mean age 49.12 ± 17.03).

Results. An increase in body weight was detected in 6.8% of children (5.4% – overweight and 1.4% – obese of the 1^st degree), 14.6 % of adolescents (11.7% – overweight and 2.9% – obese of the 1^st degree) and 62% of adults (36% – overweight, 13% – obese of the 1^st degree, 7% – obese of the 2^nd degree, 6% – obese of the 3^rd degree). During the correlation analysis, it was observed that the proportion of overweight people in the observed age categories increased with age (p < 0.05).

Conclusions. Overweight and obesity begin to be detected already in children, in a fairly large percentage of cases already occur in adolescents and are observed in more than half of the adults surveyed in St. Petersburg. Therefore, it is necessary to carry out activities among parents of preschoolers and schoolchildren, as well as, if possible, the children themselves, to form motivation to maintain a healthy lifestyle in order to prevent the early development of overweight.

研究现实性。在儿童医院革兰氏阳性菌中，葡萄球菌是化脓性脓毒性疾病的主要致病菌。耐抗生素菌株在它们中间的传播限制了治疗儿童这类感染的可能性。

本研究旨在对St.Petersburg State Pediatric Medical University 2019年门诊患者各种临床材料中分离的葡萄球菌进行物种组成特征分析，并分析其对抗菌药物的敏感性。

材料与方法。根据2018年临床建议，采用圆片扩散法860株葡萄球菌抗菌药物敏感性，采用Vitek-2全自动小型分析仪进行鉴定。

结果。院内葡萄球菌有6种，新生儿病理科和重症监护室以表皮葡萄球菌（Staphylococcus epidermidis）为主（分别为63.0和46.2%），外科和治疗科以金黄色葡萄球菌（Staphylococcus aureus）为主（分别­ 为61.7和46.2%）。超过一半的葡萄球菌（63.0%）对至少一种抗菌药物耐药。万古霉素（Vancomycin）和利奈唑胺（Linezolid）对菌株的抑制作用最强。高比例的多抗性（MDR—multidrug-resistant）培养（37.8%） 和广泛耐药（XDR—extensively drug-resistant）菌株（33.0%）被鉴定。耐药菌株以溶血葡萄球菌（Staphylococcus haemolyticus）（98.1%）和表皮葡萄球菌（S.epidermidis）（82.0%）所占比例最大。而耐药、多耐药和广泛耐药在金黄色葡萄球菌（S.aureus）中所占比例极低（分别为16,2,1,5和0.4%），以及耐甲氧西林 菌株（0.8%）。

结论。葡萄球菌的耐药谱多种多样。这类病毒在儿童医院的传播需要地方一级的持续监测。

Background. For practical health care, tools for assessing the effect of allergen-specific immunotherapy have not been developed.

Aim. Approbation of the system for evaluating the effectiveness of sublingual immunotherapy with house dust mite allergens in patients with bronchial asthma with allergic rhinitis.

Materials and methods. 28 cases of sublingual immunotherapy treatment in patients aged 5 to 13 years, (8,6 [6,7; 11,6]) with control in pairs-copies matched by age, sex, and asthma severity were analyzed. Thus, the study included 56 patients. Patients in the control group did not receive sublingual immunotherapy. For 1 year before the start of treatment, and for the first year of treatment, the complex of clinical signs of bronchial asthma and allergic rhinitis, the need for basic and emergency therapy was assessed. The scores were calculated for symptoms, for drugs, and a total Score of symptoms and drugs.

Results. During 1 year of therapy, patients showed dynamics of the total Score from 23,32 ± 1,21 points to 16,21 ± 1,77 in the main group, and from 23,99 ± 1,2 points to 20.92 ± 2.09 in control group (p = 0.028). The greatest difference was found within medication domain.

Conclusion. The developed system for assessing the symptoms and the need for medications makes it possible to show the difference between groups of patients, in favor of the sublingual immunotherapy group. For 1 year of sublingual immunotherapy therapy, a difference in the total Score dynamics and the domain of drugs was revealed between the groups. The proposed assessment system is recommended for further investigation.

COVID-19 infection usually occurs in children in a mild form, but some of them in a delayed period (one or several weeks after acute infection with COVID-19) may develop a severe inflammatory disease with clinical manifestations similar to toxic shock syndrome (Kawasaki disease), classified as multisystem inflammatory syndrome in children (MISC). It is possible that the syndrome has only a temporary connection with the COVID-19 infection. In the future, new associations of such clinical manifestations with other infectious (or non-infectious) diseases may appear. But currently, all children in the described cohorts with MISC have an association with COVID-19 infection. It is believed that the syndrome is initiated by an excessive adaptive immune response with the formation of autoantibodies. Treatment is based on anti-inflammatory, including steroid therapy, the possible use of intravenous immunoglobulin, aspirin, interleukin 1 and 6 receptor antagonists. The article analyzes current views on Kawasaki-multisystem inflammatory syndrome in children in the delayed period of COVID-19 coronavirus infection in the aspects of diagnosis, pathogenesis, clinical manifestations (with a discussion of foreign and Russian studies) and approaches to therapy and possible prevention, including the possibility of using plasmapheresis in complex therapy.

The review describes the clinical, biochemical and molecular genetic characteristics of autosomal recessive mucopolysaccharidosis type III, or Sanfilippo syndrome. This is a genetically heterogeneous group of rare, but similar in nature, diseases caused by a deficiency of one of the four lysosomal enzymes involved in the degradation of heparan sulfate. All types of mucopolysaccharidosis III are characterized by severe degeneration of the central nervous system in combination with mild somatic manifestations, which is explained by the accumulation of high concentrations of heparan sulfate in the lysosomes of various cells, including the central nervous system. The primary biochemical defect in the most common type of mucopolysaccharidosis IIIA, occurring with a frequency of 1 : 10⁵ and presented in 60% of all cases of the disease, is heparan-N-sulfatase, or sulfamidase deficiency. Mucopolysaccharidosis IIIB type occurs twice less often and accounts for about 30% of all cases of Sanfilippo syndrome. It is caused by the presence of inactivating mutations in the lysosomal α-N-acetylglucosaminidase gene. Mucopolysaccharidosis IIIC and IIID are 4% and 6%, and occur at frequencies of 0.7 and 1.0 : 10⁶. Mucopolysaccharidosis IIIC is caused by inactivating mutations in the gene of membrane-bound lysosomal acetyl-CoA:α-glucosaminid-N-acetyltransferase, or N-acetyltransferase. Mucopolysaccharidosis IIID is based on the deficiency of lysosomal N-acetylglucosamine-6-sulfatase. The role of experimental models in the study of the biochemical basis of the pathogenesis of Sanfilippo syndrome and the development of various therapeutic approaches are discussed. The possibility of neonatal screening, early diagnosis, prevention and pathogenetic therapy of these severe lysosomal diseases are considered. As an example, a clinical case of diagnosis and treatment of a child with type IIIB mucopolysaccharidosis is presented.

The differential diagnosis of paroxysmal conditions, as well as disorders of muscle tone (hypertension) in the neonatal period and in young children is quite complicated. Various states of the nervous system in newborns are transient and permanent, optimal and suboptimal, normal and pathological. Among them, we can mention non-epileptic paroxysmal states of early childhood. In some cases, non-epileptic paroxysmal states of early childhood is accompanied by motor disorders, manifested by an excessive increase in limb tone in newborns. This pathological condition of muscle tone in the English-language literature is referred to by the term stiffness baby (the syndrome of a “rigid” or “fettered” baby). Neonatal pathological muscle hypertonicity, unlike physiological hypertonicity of muscles of a newborn, is a rather rare condition. The article presents literature data and a description of the clinical observation of a patient with hyperekplexia. Hyperekplexia is a rare paroxysmal movement disorder in young children. The main clinical variants of the disease, methods of diagnosis and correction, the main mutations associated with this condition are considered. The article describes the own clinical observation of an early-age patient with hyperekplexia, its clinical picture, features of paroxysmal states and therapy, neuroimaging data, electroencephalographic phenomena recorded in the patient and genetic testing that confirmed the diagnosis of non-epileptic paroxysmal disorders. The child has a mutation in the ATAD1 gene associated with type 4 Hyperekplexia (618011).

这篇综述介绍了从Yuriy Petrovich Pushkarev教授的观点，关于血液循环和呼吸的躯体植物相互作用的 信息。这些数据说明了内稳态和适应问题的复杂性和通用性，以及许多问题的未解决性质，这些问题对于理解身体内部环境的稳定性和建立规范的边界很重要。研究表明，在人体的神经体液系统中，各种功能系统的组成部分不断地相互作用，以保证呼吸和血液循环的接口，并形成系统间的连接。在动物和人类在出生后的个体发生的功能形成的一些方面被考虑。内稳态是指体内指标的内部平衡，但其值由于中心点与周缘的功能整合而不断变化。获得有用的适应结果的过程具有生理指标的不对称性，系统间的相互作用保证了适应环境条件下内稳态常数的平衡。随着超过阈值的环境影响和内稳态参数的改变，为了维持一定水平的稳定状态，会发生补偿性和适应性反应，通过系统间连接的形成，消除或限制有害因素的作用，优化身体与环境的相互作用。对某些疾病和应激影响下机体功能改变的病理生理机制进行了分析。在Y.P.Pushkarev的思想背景下，进一步研究躯体植物相互作用，将揭示动物和人体内功能同步的真正机制，从而理解其作为一个独立的自我调节系统的存在。

This article presents the authors’ personal views on the role, the place and the forms of lecture teaching of the morphological disciplines at the medical universities. Historical analysis shows that lecture has traditionally been the leading form of a university education. At the same time, the increasing requirements for the quality and efficiency of the modern educational process, the intensive intercalation of computer technologies in education and medicine raise the question of the preferred methods of conducting this type of classes. In addition, the difficult epidemiological situation, that is observed today around the world, has made its own adjustments to this discussion. The article provides a comparative overview of the various forms of presentation of lecture material (face-to-face lectures, online lectures in synchronous and asynchronous versions), and details the advantages and the disadvantages of each of them. Evaluating the positive and the negative aspects of the remote lectures, we can say that this format is not perfect for studying at a medical university. Moreover, it is not suitable for giving lectures on human anatomy because their demonstrativeness suffers. “A lecture is a parade of a department”, – noticed the academician of the USSR Academy of Medical Sciences V.N. Shevkunenko. It can give an ability for a creativity, a lifeline in the process of a powerful flow of information to a student, select the most useful and necessary things, promote the development of а professional competence, help to find guidelines, life values and meanings. All this can be fully implemented only with “live” communication in а lecture audience, which is the secret of the longevity of a lecture.