卷 11, 编号 4 (2020)

哺乳的本能与“哺乳的时机”是影响下一代健康的关键因素在单中心观察研究新生儿围产中心的生理系的联邦国家高等教育预算教育机构Saint Petersburg State Pediatric Medical University研究了健康新生儿分娩后第一天母乳喂养的性质和困难。根据世界卫生组织的建议，对住院期间所有母婴对母乳喂养的促进和支持情况进行了分析。对103名妇女进行了社会人口学问题（年龄、婚姻状况、教育、分娩方式）、婴儿孕周、出生体重、身高、头围、阿普加评分、婴儿喂养方式的调查。只有71%的受访者是母乳喂养的，90%是按需喂养的，41.7%的妇女有母乳喂养困难。最常见的抱怨是缺乏牛奶（17.48%）和乳头皲裂（8.74%）。所有的妇女都收到了如何解决这些问题的建议。妇女对母乳喂养的认识来源的数据进行了分析，并对最近分娩的妇女提出的建议进行了分析。事实证明，有必要在哺乳母亲出院后的头几天内提供持续的专业支持。

The article is devoted to an urgent problem of pediatrics – Remheld syndrome. The aim of the present work was to study the clinical and morphofunctional features of the cardiovascular system in children with Remkheld’s syndrome. 23 children from 10 to 18 years old with Remkheld’s syndrome were examined. Research methods. Study of blood biochemical parameters (MV-CPK, ASLO titer, C-reactive protein), identification of persistent viral infection markers, ECG, daily monitoring of ECG and blood pressure by Holter, echocardiography, ultrasound of the vessels of the head and neck, ultrasound of the thyroid gland, ultrasound organs of the gastrointestinal tract. In the anamnesis, 78,3% of children had chronic gastroduodenitis, 21,7% had gastroesophageal reflux disease, and 8,7% had a hiatal hernia. All patients complained of cardialgia, interruptions in the work of the heart, a feeling of lack of air, dizziness upon admission. The appearance of noted complaints was associated with eating. Results. A study of the state of the cardiovascular system showed the presence of sinus tachycardia in 60,9% of patients, sinus bradycardia in 34,8%, single supraventricular extrasystole in 13,0%, paroxysmal supraventricular tachycardia in 4,3%, and 4,3% – autonomic sinus node dysfunction, in 13,0% – congenital heart disease (patent ductus arteriosus, bicuspid aortic valve), in 8,7% – mitral valve anterior prolapse, in 4,3% – a patent foramen ovale, in 95,7% of children – vegetative-vascular dysfunction. Conclusion. Children and adolescents with diseases of the gastrointestinal tract, when cardiac complaints appear, need an in-depth examination of the cardiovascular system.

妊娠早期诊断胎儿先天畸形和染色体异常可以降低婴儿死亡率。

目的：评价图瓦共和国无创产前检查（NIPT）的初步效果。

材料与方法。对图瓦共和国联邦国家统计局、俄罗斯卫生部和图瓦围产期中心的报告进行了回顾性分析，并对共和国医疗和遗传中心的先天性畸形监测数据进行了分析。

结果。图瓦共和国2000—2019年婴儿死亡率分析显示，指标稳步下降（29.9-7.3‰），明显超过全国平均水平。共和国婴儿死亡的主要原因之一是先天性畸形，在图瓦新生儿中发病率为12.6%-33.4%。染色体异常引起的疾病占先天性畸形的4.3-1.2%。近年来，在妊娠头三个月接受胎儿病理筛查的妇女人数有所增加（56.2-62.1%）。经联合筛查，主要染色体非整倍体（唐氏综合征、Edwards和Patau综合征）的检出率为2.1-6.6%。所有阳性筛查病例均行NIPT，特异性和敏感性较高；胎儿的染色体畸变在98.1-99.5%的病例中被证实。

结论。在图瓦共和国广泛使用NIPT将提高染色体异常检测的有效性，减少侵入性诊断干预措施的数量，避免不必要的终止妊娠和降低婴儿死亡率。

Congenital sodium diarrhea (P78.3 according to ICD 10) is a rare autosomal recessive disease, clinically and genetically heterogeneous. The sodium absorption defect is caused by disruption of the intestinal sodium-proton “pump” in the non-syndromic form and in the epithelial sodium channels in the syndromic form. Mutations in 3 genes, SPINT2 (localization 19q13.2; OMIM code 270420) – syndromic form; GUCY2C (localization 12q12.3; OMIM code 601330) and SLC9A3 (localization 5p15.33; OMIM code 616868) – non-syndromic form, can cause congenital sodium diarrhea. The frequency of the disease is unknown, since it is rare, so far only 50 cases have been described. The classic non-syndromic form of congenital sodium diarrhea is manifested by polyhydramnios, severe secretory diarrhea, severe metabolic acidosis, alkaline pH of feces >7.5 and hyponatremia. The syndrome of congenital sodium diarrhea is also manifested by choanal and/or anal atresia, hypertelorism and erosion of the cornea. Typical laboratory data include metabolic acidosis and alkaline pH of feces (fecal pH >7.5), low Na⁺ concentrations. The concentration of Na⁺ in the stool is increased. Prenatal ultrasound diagnosis allows you to identify gidroamnion and expansion of intestinal loops, starting from the third trimester of pregnancy. The diagnosis is confirmed by genetic studies. Treatment: complete parenteral nutrition with correction of water-salt metabolism. The forecast is unfavorable.

Hashimoto thyroiditis is the most common thyroid disease. This form of pathology has a diverse clinical picture, including neuropsychiatric disorders. There are frequent cases of comorbidity of autoimmune thyroiditis and psychiatric forms of pathology, along with such a nosological entity as Hashimoto’s encephalopathy (aka: Steroid-responsive encephalopathy of autoimmune thyroiditis), characterized by an increased level of antithyroid autoantibodies and various mental disorders, with still unclear pathogenesis. The question arises, how to regard patients with psychiatric disorders and Hashimoto thyroiditis — either as patients having autoimmune thyroiditis, comorbid with psychiatric forms of pathology, or as patients with Hashimoto’s encephalopathy? We studied groups of patients with autoimmune thyroiditis free from any psychiatric disorders, autoimmune thyroiditis comorbid with psychiatric forms of pathology, and a group of healthy donors similar as regards to their age and sex. We also studied medical history, clinical manifestations of the disease, instrumental data and the serum levels of thyrotropin, thyroid hormones, various antithyroid autoantibodies, and prolactin. We analyzed the correlation of laboratory and instrumental parameters and clinical data in all groups of patients. There was a significant relationship (p < 0,05) between various psychiatric symptoms and a decreased level of free thyroxine, an increased level of thyroid stimulating hormone (TSH), an increased level of prolactin and an increased volume of a thyroid gland. A significant relationship (p < 0,05) was also found between various symptoms of hypothyroidism and a decreased level of free triiodothyronine (FT3), an increased level of antibodies to thyroglobulin (anti-TG Ab), and an increased level of antibodies to thyroid peroxidase (anti-TPO Ab).

Systemic autoimmune diseases remain a complex problem for specialists of different profiles. Despite some progress in their diagnosis and treatment, the incidence of these diseases is now steadily increasing. This is primarily due to polyorganic lesion, which requires a balanced approach to the choice of treatment of the patient, taking into account the functional condition of different organs and systems. Thus in particular systemic lupus erythematosus, which occupies a leading place in the structure of rheumatological pathology, often occurs with kidneys damage, cardiovascular system, hematological disorders. Most studies are based on the systemic lupus erythematosus in women, while the male sex is recognized as a factor of adverse outcomes and severe course of the disease. The article covers the peculiarities of the course of systemic lupus erythematosus in men, considered the variants of heart affection within the systemic process, and also presents a clinical case of systemic lupus erythematosus of high activity in a young man with terminal kidney affection, pancarditis with formation of severe mitral insufficiency, pancytopenia. The aspects of the management of a patient with severe valve disease of the heart in need of renal replacement therapy against the background of drug immunosuppression, the possibility of correction of valve disease. The combination of terminal renal failure and heart failure in a young man in the clinical case cited is an example of the multidiscplinar problem of treating systemic lupus erythematosus.

这篇文章致力于研究经历与足部截肢的老年患者的危机的特殊性。我们研究了31例年龄为59-73岁有截肢危险的患者，他们在重建手术阶段正在医院接受治疗。第二组由32名年龄为59-73岁的患者组成，他们至少在7天前接受过截肢手术，目前正在医院进行术后康复。研究方法：实证研究（访谈、问卷调查、测试）。对实证研究过程中获得的各项指标进行比较，发现研究组之间存在若干差异，即有截肢威胁的老年人更容易经历负面情绪体验，使用适应不良的应对策略，对不良态度的严重程度有较高的指标，对自己的身体感到不满，并将负面情绪体验与下肢，尤其是双脚联系起来。与有截肢危险的病人相比，截肢的病人更有可能体验到积极的情绪，注意到较少的明显的疼痛，使用《理智化》防御机制，较少注意到对整个身体的不满意，并把整个情绪范围投射到头部区域。介绍了心理矫正方案对足部截肢威胁患者心理情绪状态优化效果的测试和检验结果。

The pathology practice environment varies per healthcare setting. However, anatomic pathology is a visual applied science discipline and incorporation of high-quality images into a surgical pathology report is essential. Each specimen received for morphological examination is unique and variation in the description can exist between prosectors and they experience. That is why gross descriptions supported with digital photographs can eliminate the insufficiency of macroscopic examination. To form and strengthen pathologists’ competencies in digital macroscopic photography a problem-based learning approach is used for training. A problem-based learning ensures the strength of the acquired knowledge since it is obtained in an independent activity. The article discusses what type of problems a pathologist should solve when taking a macroscopic photograph of a surgical specimen. An analysis of literature on modern equipment for digital macroscopic photography was performed. Recommendations for step-by-step photographing, and schematic mapping for surgical specimen triaging are provided. An option is proposed for actively developing professional competencies including creation of digital photo archives of surgical gross specimens, as well as study sections and discussions by professionals at forums such as society meetings. It was concluded that pathologists’ competency in digital macroscopic photography is necessary to maintain a high standard of medical care.

The editorial presents medical, scientific, pedagogical activity academician of the USSR Academy of Medical Sciences, Honored Scientist of the RSFSR Mikhail Stepanovich Maslov (1885–1961). Mikhail Stepanovich Maslov Founder and Head of the Pediatrics Department of the Leningrad pediatric medical Institute (1930–1961). Academician of the USSR Academy of Medical Sciences Michael Stepanovich Maslov has made a great contribution in the development in domestic pediatrics. The head of the Department of faculty Pediatrics of LPMI, аcademician M.S. Maslov went down in history as a great scientist, one of the founders of the Russian scientific pediatric school, as a teacher who brought up several generations of pediatricians and scientific personnel, as a doctor who restored the health of hundreds of thousands of sick children. Academician M.S. Maslov is the author of more than 200 scientific works, including 16 monographs and 8 textbooks. Under the guidance of academician M.S. Maslov, 36 dissertations of the candidate of medical Sciences were completed and defended, 10 dissertations of doctors of science were prepared with scientific advice. The great scientist, teacher, doctor and public figure Mikhail Stepanovich Maslov is the pride of Russian pediatric science, education and healthcare.