卷 11, 编号 5 (2020)

Introduction. Small heart anomalies (SHA) are the morphological basis for functional changes in cardiac activity and can exacerbate the course of organic heart lesions. The most studied SHA include false chords of the left ventricle (FCLV) and mitral valve prolapse. Prevalence, association with external signs of dysembryogenesis, as well as the predictive value of SHA are not sufficiently studied.

Materials and methods. We examined 611 people between the ages of 18 and 23 (average age 20.3 ± 1.6 years), including 257 boys and 354 girls. All of the surveyed performed phenotypic, anthropometric and echocardiographic examinations. To identify the SHA links to heart rhythm disorders, the 205 surveyed performed Holter’s ECG monitoring.

Results. SHA identified in 90% of the individuals surveyed: atrial septum aneurysm (24%), tricuspid valve prolapse (23.4%), asymmetry of the aortic valve (20.6%), additional papillary muscles (39.4%) and FCLV (75,1%). Correlation analysis showed the presence of links between these SHA and bone signs of dysembryogenesis (chest deformities, arachnodactyllia, dolistennomely and high palate), as well as heart rhythm disorders (supraventricular and ventricular extrasystoles, rhythm driver migration and episodes of AV-blockade 1 degree). Patients with marfanoid habitus have a higher average number of SHA (2.1 ± 1.4 vs 0.9 ± 0.7, p < 0.005).

Conclusions. SHA are identified in the vast majority of healthy people. Bone signs of dysembryogenesis are associated with significant SHA and can serve as a marker for the involvement of the heart in the dysplastic process. Patients with SHA have significant cardiac arrhythmias.

The article is devoted to the analysis of mental, including speech, disorders among children in the villages of the Srednekolymsky ulus of the Republic of Sakha (Yakutia). The purpose of the survey was to analyze the diagnosed mental, including speech, disorders among the children of the villages of the Srednekolymsky ulus of the Republic of Sakha (Yakutia). 501 children aged 2 to 18 years were examined. Clinically significant disorders were detected in 187 children (37.4%). The structure of mental disorders is described: the first place is occupied by specific speech and language disorders (F80), the second is mental retardation (F83) and the third is mild cognitive impairment (F06.7). The data on the prevalence of mental disorders in various age groups are presented: most often, mental disorders occur in adolescence (51.0% of all examined children). Less disturbances are diagnosed in the early age group and the first period of childhood – 22.0% of all children examined. In the group of preschool age, speech disorders are in the lead, in the group of primary school age – mental retardation, speech disorders and mild cognitive impairment. In adolescence, a wide range of mental disorders is diagnosed, including adaptive reactions and eating disorders. Sexual differences in the nosological specificity of mental disorders were revealed: mental disorders prevail in boys. Speech disorders, stuttering, and behavioral disorders are significantly more common among boys. Among girls, adaptation disorders, emotionally labile disorders and eating disorders are significantly more common.

The review presents data on two similar granulomatous inflammatory diseases: tuberculosis and sarcoidosis of the lungs, which together cover about 5% of all pulmonary pathology, albeit occur with different incidence (20 : 1). Despite the established aetiology of tuberculosis, the disease has not disappeared and nowadays has even acquired a new urgency: It is getting out of control due to growing poverty, the comorbidity with HIV infection, increasing cases of drug resistance of Mycobacteria, insufficient effectiveness and the growing costs of its treatment. Against the background of the expansion of anthropogenic influences and other environmental impacts on the immune system, the incidence of lung sarcoidosis is also increasing, while patients are initially often misdiagnosed with tuberculosis, with resulting unjustified anti-tuberculosis chemotherapy, leading to chronization of the disease with frequent relapses and, accordingly, to an increase in disability and mortality rates. In recent years, clinical manifestations of sarcoidosis due to a variety of trigger aetiological factors with adjuvant-like action (from Mycobacteria to xenobiotics) – are considered by a number of authors as a variant of autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA). The article emphasizes the similarity of two granulomatous inflammatory diseases and the concept of two variants of the body’s response to similar or even identical aetiological factors within different human reactivity (possibly on a different mosaic/permissive background). In brief the newest data on experimental models of sarcoidosis are reviewed as well as the role of autophagy disorders and opposite macrophageal polarization in tuberculosis versus sarcoidosis. Authors coined the original hypothesis of the possible therapeutic effectiveness of Rapamycin in sarcoidosis and for the first time posed a question of equivocal character of comorbidity between these granulomatoses and COVID-19 infection.

Hemangiomas are the most frequent tumors of childhood age. In the first year of life their prevalence is 10–12%. Among premature children, the prevalence of hemangiomas correlates with the degree of prematurity. Liver hemangiomas are a wide range of benign vascular formations that can acquire both malignant course and capable of spontaneous regression. Small-sized hemangiomas generally do not require specific treatment and proceed asymptomally. Large-sized formations can cause life-threatening conditions such as severe thrombocytopenia with coagulopathy (Kazabah–Merrit syndrome), anaemia, tumor hemorrhage, spontaneous and traumatic tumor breaks, heart failure associated with intra-tumor bypass of blood flow, abdominal compression syndrome, severe hypothyroidism. Diagnosis of this pathology is carried out on the basis of a set of data of medical imaging, laboratory diagnostics and clinical picture. This review presents key points of general classification, the most characteristic diagnostic signs, as well as basic algorithms of treatment of liver hemangiomas in newborns and children of the first year of life. There are also described 2 clinical cases of newborns with giant liver hemangiomas, which were examined and treated in the pathology department of newborns and infants of Perinatal Center of St. Petersburg State Pediatric Medical University with a description of the peculiarities of the course of each of them.

排尿困难的原因可以是不同的，并且在一般的医疗实践中，心因性排尿困难的案例不被认为是诡辩。这些病人主诉排尿增多（频尿）、尿失禁（遗尿）、尿潴留或排尿感觉丧失。通常情况下，这类患者第一次求助于儿科医生（治疗师），然后求助于泌尿科医生，后者在经过全面检查后排除了任何对泌尿生殖系统的器质性损伤。精神性排尿困难可以在一般神经症的结构中观察到，是一种躯体形式的障碍或神经症（通常转化）的单症状。歇斯底里症（转换）的定义不是装病的，而是操纵的，也就是说，作为一种吸引人们注意的方式，或者，从病人的角度来看，这是一种走出绝望境地的无意识方式，就像上述案例中的情况一样。出于同样的原因，癔症患者的治疗仍然是最困难的问题之一，需要专业专家的参与—心理治疗师。在这种情况下，综合心理治疗（理性、行为、暗示）是相当有效的，但通常需要长期的治疗才能达到良好的效果。给定的临床例子是科学和实际的兴趣，因为充分的效果是在第一次咨询和催眠暗示治疗后实现的。

分析了“心理健康规则”概念的各种方法和模型：医学（生物学）、心理学、社会规范，基于机械决定论的原则。A.B.Kholmogorova提供了基于系统方法的生物心理社会模型。B.S.Bratus，批判性地评价了“心理健康规则”定义的这些模型和方法，作为另一种方法强调了人的哲学概念。这允许理解人的本质及人的发展。因此，寻找“心理健康规则”标准的问题可以重新表述为寻找“人心理发展规范”标准的问题，由于重点是人类作为文化发展的所有多样性的结果：作为一个个体，一个人，一个活动的主体，其许多属性的综合表现为个体性。人在文化框架内的发展导致文化意识的形成，文化意识的主要指标是自由、独立、创造性、自给自足和自觉。试图为实现人类心理发展的目标而制定心理工具。在个体人格层面上，这一角色属于个体取向，决定了其对活动目标实现的着重点；在活动主体层面上，这一角色属于个体活动风格。这些人的发展主导因素的相互作用，保证了人的组织各层次的内在统一，形成了“人的完整性”概念，充分表达了文化意识载体—个体的心理内涵。

俄罗斯遗传学形成早期阶段的显著特征包括对优生学的明显兴趣，细胞遗传学领域的优先成就，研究遗传对多因素疾病，人的生理和心理特征发展的贡献的程序系统。到20世纪30年代中期发展起来的强大的遗传学科学基础设施在该国领导层的一系列政治决策中遭到破坏：从根除优生思想的运动开始，到1948年Lenin All-Union Academy of Agricultural Sciences会议结束，其导致了遗传学研究的全面禁止。在近四分之一个世纪的时间里，俄罗斯遗传学研究的进展一开始完全停滞，然后极其困难。医学遗传学的复兴有多种途径，依靠人类遗传学各个领域的许多杰出专家的工作，他们的科学兴趣甚至在压抑时代之前就形成了。他们建立了一所新的科学学院。尽管俄罗斯与世界遗传学水平存在着巨大的差距，而且在禁令解除后的数年里仍然存在着巨大的差距，但在辐射诱变、种群遗传学、细胞遗传学等领域的一系列重要工作值得注意，以及在生物化学遗传学和代谢性疾病研究领域取得了多项世界一流的成果。国家逐渐认识到遗传学对医学的重要性，在卫生体系中形成了广泛的医学和遗传学服务。医学遗传学最终恢复的条件点可以被认为是80年代末在俄罗斯医科大学出现的这一学科的院系。尽管俄罗斯的医学和遗传科学仍然落后于世界水平，但可以说，到目前为止，这一差距已有所缩小。