卷 12, 编号 3 (2021)

Background. The diagnosis of a woman in labor “chorioamnionitis” (CA) implies a high risk of infectious complications for the mother and fetus, which determines the need for additional examination of infants and the decision on the appointment of antibacterial therapy. The purpouse of this study was determine the need to administration antibiotic therapy to full-term newborns from mothers diagnosed with chorioamnionitis.

Materials and methods. 113 full-term newborns were examined, of which the main group consisted of children whose mothers were diagnosed with “chorioamnionitis” (n = 77), the comparison group – children born to healthy mothers (n = 36). All children performed clinical and laboratory monitoring, including a clinical analysis of capillary blood in the first 24 hours of life; determination of the level of C-reactive protein (CRP) in venous blood on the 3rd day of life. Bacteriological examination of newborns included sampling of material from the ear fold, buccal mucosa, umbilical cord blood, as well as the contents of the tracheobronchial tree (TBD) – during respiratory therapy with mechanical ventilation. Special research methods included studies of the proinflammatory cytokines (IL-1β, IL-6) in umbilical cord blood. Histological CA was diagnosed in the presence of morphological and functional signs of inflammation in the placenta.

Results. Newborns of the main group more often developed respiratory disorders requiring respiratory and oxygen therapy (p = 0,045). The production of IL-1β, IL-6 in umbilical cord blood in the examined newborns of the main group was higher than in the comparison group [Odds Ratio (OR) 8.4; 95% Confidence Interval (CI): 1.0–67.9; OR 7.4; 95% CI: 2.5–21.7 respectively]. The study of blood samples revealed leukocytosis (>34·10⁹) 6.5% vs 0%, p > 0.05) and a shift in the leukocyte count to young forms of neutrophils (45.4% vs 16.7%, p < 0.05) in the peripheral blood of infants of the main group. Infants exposed to maternal clinical chorioamnionitis had increased level of CRP 10.3 times more frequent (95% CI: 2.8–37.1) than in newborns in the comparison group. With dynamic clinical and laboratory monitoring, 72 children of the main group (93.5%) had no data for the course of the infection, as a result of which they did not receive antibiotic therapy.

Conclusion: Administration antibiotic therapy to clinically healthy full-term newborns from mothers diagnosed with chorioamnionitis is unjustified. Infants of this group require clinical laboratory, dynamic observation with laboratory control, including a clinical blood test and determination of the CRP level, which is a preferred alternative to the appointment of antibiotic therapy.

绪论在血液学中，迫切需要解决的问题之一是成血细胞增多。现代治疗方法已明显改善了成血细胞增多症的预后。然而，化疗的使用伴随着高频率的药物并发症，包括那些与神经毒性有关的。在疾病的主要临床症状之外加上神经系统症状会显著加重患者病情的严重程度，影响预后和生活质量。本文介绍急性淋巴细胞白血病患儿化疗引起的多神经病变的神经生理学特点。

本研究的目的是比较急性淋巴细胞白血病患儿化疗引起的多神经病变的临床表现和神经生理征象。

材料与方法。以The Regional Children's Clinical Hospital of Yekaterinburg在2019–2020年为基础， 对21例年龄为3至17岁儿童的临床图像进行分析并评价神经电图（ENMG）研究。

结果。在儿童研究组中，根据神经生理学研究，几乎所有患者（95.2%）在诱导化疗阶段就确定了周围性多神经病变的体征。腓骨神经运动轴突神经病的优势被揭示。同时，25%的患者记录了临床表现。在4个月的动态随访中，所有有周围神经损伤神经生理体征的儿童都出现了相应的神经症状。根据ENMG的研究结果，出现了消极的趋势。混合型多神经病变患者增加了1.7倍。在每三分之一的儿童中，M波的振幅和冲动传导速度的速率都有所下降。

结论。所获得的数据决定了需要尽早采用神经生理学方法进行研究，以确定急性淋巴细胞白血病患儿接受化疗时神经毒性并发症形成的高危人群，并及时预约一套复杂的治疗措施。

Among the manifestations of Hashimoto’s autoimmune thyroiditis, there are various psychoneurological disorders. For more than a century, it has been known about psycho-neurological disorders associated with hypothyroidism, but along with that, there are also mental disorders in patients with thyropathies in euthyroid state. In 1966, Hashimoto’s encephalopathy was described, the pathogenesis and clear differential diagnostic criteria of which have not yet been determined. This article describes an experimental study in laboratory mice with intracisternal stereotaxic injection of IgG isolated from patients with autoimmune thyroiditis and comorbid depression or schizophrenia. A control group included animals receiving polyclonal IgG from healthy donors. Then behavioral tests were carried out, which revealed the characteristics and changes in behavior in the operated animals. Thus, animals that received immunoglobulins from patients with autoimmune thyroiditis and depression were less active in relation to the development of risk behavior. Porsolt’s tests on the 4th and 15th days after surgery showed that, regardless of the kind of the injected solutions, there was a change in the temporal relationships between the behavior patterns. In mice received IgG from patients with autoimmune thyroiditis and schizophrenia during the delayed Porsolt test, the ratio of the forms of motor activity shifted towards passive swimming. The mice received IgG from healthy donors did not demonstrate this change.

儿科医生对血液中乳酸水平的评估有时会造成困难。乳酸盐是葡萄糖、脂肪酸和一些可互换氨基酸的细胞代谢产物。血液中的乳酸标准不是由国际标准定义的，因此它取决于实验室中使用的方法学和试剂。血液中的乳酸水平是其形成和利用过程之间平衡的结果。导致血乳酸增加的主要原因是无氧肌肉活动（剧烈运动、癫痫）、肺部和心脏疾病（呼吸衰竭、循环功能不全）、肝肾功能受损、糖尿病、败血症、休克和新生儿一些严重异常、线粒体疾病、药物、维生素不足。诊断和预后解释血乳酸水平的可能性还没有用尽。乳酸酸中毒是由某些病理条件（疾病或综合征）引起的高乳酸血症的危险并发症，可导致死亡。确定乳酸酸中毒的原因是选择治疗策略的关键因素。乳酸性昏迷是一种罕见但可能的糖尿病并发症，需要在重症监护病房进行强化综合治疗。儿科医生应该能够评估血液中的乳酸水平，分析高乳酸血症的可能原因，并知道乳酸酸中毒发展的行动算法。

Mucopolysaccharidosis (MPS) are a genetically heterogeneous group of rare monogenic metabolic diseases associated with hereditary insufficiency of lysosomal enzymes involved in the catabolism of glycosaminoglycans, or mucopolysaccharides. The pathogenesis of MPS is due to the accumulation of non-cleaved glycosaminoglycans in lysosomes, which can destroy cells. All MPS are characterized by a polysystemic manifestation, the simultaneous involvement of many organs and tissues in the pathological process, first of all, connective tissues, bones and cartilaginous. This review presents the epidemiology, clinical, biochemical, and molecular genetic characteristics of MPS types I and II, caused by the recessive mutations in the alpha-L-iduronidase and iduronate-2-sulfatase genes, respectively, and by the accumulation of dermatan and heparan sulfate. Each of these diseases is characterized by clinical polymorphism, especially observed in MPS I, which often manifests in a severe form of Hurler syndrome, but can also occur in a milder form of Scheie syndrome. Currently, there is an increased interest in MPS in the world due to the identification of the spectrum and frequencies of mutations in the IDUA and IDS genes in various populations, including in Russia, and the practical availability of methods for individual molecular diagnostics. The description of the existing experimental models, their role in the study of the biochemical basis of the pathogenesis of these severe hereditary diseases and the development of various therapeutic approaches are given. Discusses the possibility of early diagnosis of MPS I and II types based on neonatal screening in order to increase the effectiveness of their prevention and treatment, as well as the advantages and disadvantages of the main approaches to the treatment of these serious diseases, such as hematopoietic stem cell transplantation, enzyme replacement and substrate-reducing therapy. A clinical example of a combination therapy for a severe form of mucopolysaccharidosis type I – Hurler syndrome is presented

本文对不孕症患者的应激状态进行了研究。研究不孕症应激的焦虑、抑郁、应对策略、情绪计划等因素，以及生活质量的心理因素。摘要本研究的目的在于探讨不孕症压力的个体成分，如焦虑、抑郁、患者的情绪模式和应对压力的方法。研究方法选择：A.Beck焦虑量表、A.Beck抑郁量表；FertiQoL J.Boivin（不孕症患者）生活质量调查问卷；《R.Leahy's的情绪计划量表》（LESS II）问卷；T.O.Gordeeva，E.N.Osina， E.A.Rasskazova，O.A.Sychev，V.Y.Shevyakhova对适应中COPE应对策略的问卷调查。一项研究对33名不孕妇女（主要组）和33名没有不孕而且近期不打算怀孕的妇女进行了研究。研究结果揭示了不孕妇女对生活质量的满意度不高、主观上的情感不适、社会支持不足以及对婚姻关系的不满意等特征。与此同时，亚临床水平的焦虑抑郁症状伴随着普遍紧张的情绪计划，《对自己的情绪感到内疚》，《情绪没有足够的意义》， 《他人情感上的缺陷》，《情绪预测持续时间》，《沉思倾向》，《情绪的简化观念》计划占主导地位。不孕症压力女性在压力情境下的行为策略表现为缺乏灵活性、回避和否认倾向、很少使用积极的重新表述和幽默。研究揭示了不孕压力的各种支持因素的存在，这是不孕女性的特点：对生活质量满意度不高，主观感受情绪不适，社会支持不足，对婚姻关系不满意。与此同时，亚临床水平的焦虑-抑郁症状伴随着普遍紧张的情绪计划和有限的行为策略在压力情况下。

The article presents the medical, scientific, and pedagogical activities of the Honored Scientist of the Russian Federation Albert Vazgenovich Papayan (1936–2002), Head of the Department of Faculty Pediatrics (1974–2002), Dean of Foreign Students (1969–1985), Vice-Rector for International Relations (1999–2002) of the Leningrad Pediatric Medical Institute, then the St. Petersburg State Pediatric Medical Academy. Professor A.V. Papayan is an outstanding scientist who formed the largest pediatric nephrology school within the walls of the SPbSMA, as the founder of the study of the hemostasis system in kidney diseases in children, as a teacher who brought up several generations of pediatricians and nephrologists, as a doctor who restored the health of thousands of sick children. Professor A.V. Papayan is the author of over 400 scientific papers, including 14 monographs and chapters in 14 manuals. Under the guidance of Professor A.V. Papayan completed and defended 62 dissertations of the Candidate of Medical Sciences, with scientific advice – 1 dissertation of the Doctor of Medical Sciences. Honored Scientist of the Russian Federation Albert Vazgenovich Papayan is the pride of the national pediatric science, education and health care. The editorial presents medical, scientific, pedagogical activity academician of the Russian Academy of Medical Sciences, Honored Scientist of the Russian Federation.