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Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases characterized by a defect in one of the enzymes or transport proteins involved in the cortisol synthesis in the adrenal cortex. The most common form of CAH, which occurs in more than 90% of cases, is a 21-hydroxylase enzyme deficiency. The latter is subdivided into nonclassical and classic (salt-losing and virilizing) forms. The prevalence of classic forms of 21-hydroxylase deficiency ranges from 1: 14,000 to 1:18,000 live births worldwide. According to the data of neonatal screening in the Russian Federation, the prevalence of the disease in some regions ranges from 1: 5000 to 1: 12000, in the country as a whole - 1: 9638 live newborns. The non-classical form of CAH occurs more often - from 1: 500 to 1: 1000 among the general population. In second place is the hypertensive form of CAH - a deficiency of 11β-hydroxylase, which, according to the literature, occurs in about 1 per 100,000 newborns. These clinical guidelines were compiled by a professional community of narrow specialists, approved by the expert council of the Ministry of Health of the Russian Federation, and updated the previous version published in 2016. The clinical guidelines are based on systematic reviews, meta-analyses and original articles, and scientific work on this issue in the Russian Federation and other countries. The purpose of this document is to provide clinicians with the most up-to-date, evidence-based guidelines for the CAH diagnosis and treatment

Background: Aldosterone-producing adrenocortical adenoma (APA) is responsible for the majority of cases clinically diagnosed as primary aldosteronism. Aldosterone synthase (CYP11B2) is one of the enzymes that play essential roles in aldosterone synthesis and is involved in the pathogenesis of primary aldosteronism. Recent studies have demonstrated that various factors influence the expression and function of CYP11B2 in APA. In particular, somatic mutations, such as gain-of-function and loss-of-function mutations have been identified in several genes, each of which encodes a pivotal protein that affects the calcium signaling pathway, the expression of CYP11B2, and aldosterone production. On the other hand, CYP11B2 also catalyzes the conversion of cortisol to 18-hydroxycortisol and subsequently converts 18-hydroxycortisol to 18-oxocortisol. The article also discusses the clinical significance of 18-oxocortisol, an important biomarker for the diagnosis of primary aldosteronism. Somatic mutations in aldosterone-driver genes are strongly associated with CYP11B2 expression and have been only detected in the CYP11B2-positive tumor area, that indicating heterogeneous expression of CYP11B2 in tumor.Aim: to assess the functional heterogeneity of adrenal tumors in primary aldosteronismMaterials and methods: Retrospective evaluation adrenal tumors from patients with primary aldosteronism (n=20). According to CT unilateral macrohyperplasia was detected in 19 patients (95% of total), all of them were confirmed to have unilateral hyperproduction of aldosterone according to AVS. Selected tumors werestained with anty-CYP11B2 antibody. We evaluated the CYP11B2 expression in the adenoma and in the adjacent adrenal cortex.Results: Immunohistochemistry studies of the resected adrenals from 20 patients with PA operated due to unilateral production of aldosterone using CYP11B2 staining showed that 10 of those with an adenoma on CT scanning showed CYP11B2 staining in the adenoma. Furthermore, 5 cases of an unilateral adenoma, showed CYP11B2 staining in the adjacent adrenal cortex and an absence of staining for CYP11B2 in the adenoma. 5 cases showed CYP11B2 expression is heterogeneously immunolocalized throughout the tumor area.Conclusion: Thus, the functional heterogeneity of adrenal tumors in primary aldosteronism has been proven. It is necessary to compare the data of immunohistochemical studies on the expression of CYP11B2 with the indicators of the level of 18-hydroxycortisol, 18-oxocortisol.

Introduction. In Russian Federation, there are no comprehensive studies assessing the quality of life and risk factors for vitamin D deficiency and insufficiency, taking into account its status in different geographic latitudes.Aim. To assess the quality of life and risk factors for vitamin D deficiency and insufficiency among the population living in the regions of the Russian Federation located at latitudes from 45 ° to 70 °.Materials and methods. The first stage of the Russian multicenter non-interventional registry study using the “cross-sectional” method was carried out from March 2020 to May 2020.Results and discussion. According to the results of the correlation analysis, qualitative and quantitative factors were identified, presumably being risk factors for vitamin D deficiency and deficiency. Qualitative risk factors include: education; alcohol consumption; being in direct sunlight for more than 30 minutes a day; visit to the solarium; using sunscreen; drinking coffee; taking medications (not vitamin-mineral complexes). Quantitative factors include: visits to specialists (total per year); smoking (duration, years); exercise for more than 30 minutes a day, once a week; being in direct sunlight for more than 30 minutes a day.Conclusion. A wide range of risk factors for vitamin D deficiency dictates the need for their further study to clarify the category of persons who are shown targeted biochemical screening with subsequent drug correction.

Pheochromocytoma/paraganglioma is a neuroendocrine tumor of chromaffin and nonchromaffin cells of the autonomic nervous system, in most cases localized in the medullary layer of the adrenal gland. Its development is often associated with genetic predisposition. More than 30% of adult patients have genetically determined PCC/PG. In the last decade, many genes predisposing to the manifestation of PCC/PG have been found: RET, VHL, NF1, SDHB, SDHC, SDHD, SDHA, SDHAF2, TMEM127, MAH, KIF1BP, PHD2, EGLN1, FH, H-RAS, IDH, SLC25A11, MDH2. Mutation of the oncosuppressor genes TMEM127 and EGLN1, which regulate the level of factors induced by HIF hypoxia, is extremely rare in patients with PCC/PG, and has not been described at all in combination with pancreatic tumor. To date, data on the clinical manifestations of these gene mutations are limited. Accumulating clinical data on patients with identified genetic alterations is important for predicting the course of the disease, clarifying the malignant potential and stratifying the risk of developing comorbid pathology. We present the clinical case of a 62-year-old patient with PCC/PG and pancreatic tumor in whom a previously undescribed combination of TMEM127 c.99G > A (p.S33S) and EGLN1 c.515C > T (p.A172V) gene variants was found.

Introduction. One of the objectives of weight loss in obesity is to prevent metabolic disorders associated with it. An important component in the maintenance of the achieved results is a change of eating behavior.Goal: to study the effect of liraglutide 3.0 mg on the dynamics of metabolic parameters and eating behavior in patients with obesity. Materials and methods. The study enrolled 42 obese patients in whom anthropometric parameters, metabolic parameters, and eating behavior were assessed with Dutch Eating Behavior Questionnaire (DEBQ). Patients were divided into 2 groups, one of which received liraglutide 3.0 mg with lifestyle modification for 3 months. The other group was recommended to receive only lifestyle modification. The participants were re-examined after 3 months.Results and discussion. in the liraglutide group in addition to a significant decrease in body weight, BMI and waist circumference, there was a statistical trend toward lower glucose, insulin and HOMA-IR levels. When comparing the dynamics of parameters between the groups, Д body weight, BMI and glucose in the liraglutide group were significantly superior. In reassessment of eating behavior after 3 months of treatment, no statistically significant differences were found with the initial severity of restrictive, emotional, and/or external types in both groups and, despite a more pronounced decrease in body weight in the liraglutide group, between them.Conclusions: Three months of isolated lifestyle modification and/or its combination with liraglutide 3.0 mg is not sufficient to make a lasting change in eating behavior. However, considering that obesity is a chronic and relapsing disease, the need for eating behavior correction remains relevant to prevent disease recurrence. This substantiates the need for more long-term intervention in obesity, including drug therapy.

Riedel's thyroiditis is a rare disease characterized by chronic fibrosis. Clinical performance of this disease is dense stony goiter, which can poorly be displaced during palpation. The overgrowth of the goiter can lead to the development of compression syndrome. To diagnose we need to made fine needle biopsy and made the final diagnose according to its results or according to the morphological description of the postoperative material. An important step in the diagnosis of Riedel's thyroiditis is the determination of serum IgG and IgG4 to exclude an IgG4-associated disease. Treatment of this disease includes drug therapy, which is based on glucocorticosteroids administration or surgical treatment when develops compression syndrome. This article presents a clinical case of a patient with Riedel's thyroiditis; the main complaints were associated with the growth of goiter and the development of compression syndrome. In this regard, patient underwent surgery on the thyroid gland, and after this we get final diagnose. Due to feeling unwell, drug therapy with glucocorticosteroids was prescribed, against the background of which we noted a positive trend.

The nature of human nutrition has become increasingly important as an effective element in the prevention and treatment of many pathologies, especially obesity, type 2 diabetes and cardiovascular diseases. High protein diets are some of the most popular eating patterns and the Dukan diet has taken the lead in popularity among the diets of this type. An increase of protein in the diet is effective in reducing body weight, primarily due to the loss of adipose tissue, without a significant effect on muscle mass. Another advantage of a high-protein diet is earlier and longer satiety compared to other diets, which makes it comfortable for use. Besides obesity, high protein diets are presumably effective for treating such diseases as nonalcoholic fatty liver disease, diabetes mellitus and cardiovascular diseases However, despite the important advantages, this nutritional model is not universal and is contraindicated in patients with diseases of liver, kidneys and osteoporosis. Besides, the prolonged use of a high protein diet may increase the risks of urolithiasis and reduced mineral bone density even for healthy individuals. Thus, the increase in the proportion of protein in the diet should take place exclusively under the supervision of a physician.

Thyroid disease, including autoimmune genesis, and diabetes mellitus are the two most common endocrine diseases in clinical practice. These pathologies have a mutual influence on each other. On the one hand, thyroid hormones are involved in regulation of carbohydrate metabolism: excessive production of thyroid hormones is associated with hyperglycemia, while hypothyroidism has a decreased level of glucose production by the liver. On the other hand, glucose homeostasis disorder affects the functional state of the thyroid gland: dystrophic, sclerotic and atrophic processes are developing in thyroid gland, what is a manifestation of diabetic microangiopathy. This article demonstrates the need to take into account the mutual influence of pathologies for optimal treatment of this conditions.

The prevalence of hypothyroidism in the population is high. The frequency of manifest hypothyroidism in the world, according to various data, is 0.2–2.0%, subclinical one – up to 10% for women and up to 3% for men, and in the older age group (over 70 years) reaches 14%, with the majority of cases of hypothyroidism accounted for primary hypothyroidism. Thus, a doctor of any specialty in his practice is likely to meet a patient with hypothyroidism: both with the established diagnosis, and face the need for differential diagnosis of various pathological conditions with hypothyroidism. This article presents a classification of hypothyroidism based on etiological aspects, describes the clinical picture of the disease, pays special attention to the so-called «masks» of hypothyroidism, which, in our view, can be useful for a doctor of any specialty, provides available methods for diagnosing this syndrome (special attention is paid to laboratory methods), as well as the goals and principles of treatment, highlights the need to monitor laboratory indicators in dynamics against the background of treatment. In addition, the features of correction of hypothyroidism during pregnancy are given. The article presents the peculiarities of selecting drug doses depending on the patient’s age and comorbidity. The distinctive feature and the purpose of this article, from our point of view, is its potential benefits not only for endocrinologists, but also for other health professionals.

Introduction. Lumbosacral radiculopathy is а leading cause of long-term disability. Taking into a consideration the duration of treatment radiculopathy, the risk of developing adverse reactions when taking analgesics, non-steroidal anti-inflammatory drugs, the physiotherapeutic method — repetitive peripheral magnetic stimulation may become a promising method of therapy.Aim of the study. Assessment of the effectiveness of the complex treatment for patients with lumbosacral radiculopathy using the course of the repetitive peripheral magnetic stimulation.Materials and methods. Forty patients with lumbosacral radiculopathy were enrolled in the open non-randomized study, were divided into 2 parallel groups. The patients of the 1st group received a course of traditional treatment and a course of the repetitive peripheral magnetic stimulation. The patients of the 2nd group were treated with the traditional treatment without the course of the stimulation. A magnetic stimulator MagPro (Magventure, Denmark) was used for repetitive peripheral magnetic stimulation.Results. A significant difference (p 0.05) in pain syndrome, functional status, anxiety level at the end of follow-up between the groups.Conclusion. We did not receive the benefits of the repetitive peripheral magnetic stimulation course in comparison with a traditional treatment of a lumbosacral radiculopathy. Further placebo-controlled studies to study the effect of repetitive peripheral magnetic stimulation on pain and anxiety in patients with back pain and radiculopathy are required.

Primary hyperaldosteronism (PA) - is the clinical syndrome, results from autonomous of the major regulators of secretion, aldosterone overproduction by a tumorous or hyperplastic tissue in adrenal cortex. Being the most frequent cause of secondary hypertension, PA may be represented by disorders with unilateral or bilateral aldosterone overproduction and differential diagnosis between them is crucial for choosing a right therapeutic approache: lifelong medical therapy with mineralocorticoid receptor antagonists or unilateral adrenalectomy. Adrenal venous sampling (AVS) is currently the «gold standard» test for identifying laterality of excess hormone production, unlike imaging tests, sensitivity and specificity of which is not enough, due to inability to evaluate functional activity with confidence, and also to limitations in detecting tiny abnormalities of adrenals, such as microadenoma or hyperplasia. Excluding certain cases, AVS is recommended to patients with confirmed PA, planning surgical treatment, to determine the lateralization of aldosterone hypersecretion. Described clinical case of patient with confirmed lateralization from adrenal without any detected lesions on CT-imaging and nonfunctioning tumour on contralateral side, highlights the importance of using AVS for decision to refer patients for surgery.Первичный гиперальдостеронизм (ПГА) - клинический синдром, развивающийся в результате избыточной секреции альдостерона функционально автономным от ренин - ангиотензин - альдостероновой системы опухолевым или гиперпластическим процессом в коре надпочечника. Являясь крайне распространенной формой вторичной артериальной гипертензии, ПГА может быть представлен нозологиями как с односторонней, так и с двусторонней гиперпродукцией альдостерона, дифференциальная диагностика между которыми является ключевым моментом для определения лечебной тактики и показаний к оперативному лечению, целесообразному лишь при одностороннем варианте ПГА. В настоящее время сравнительный селективный венозный забор крови (ССВЗК) из надпочечниковых вен является «золотым стандартом» в дифференциальной диагностике нозологических форм ПГА, в отличие от визуализирующих методов, не обладающих достаточной чувствительностью и специфичностью. За исключением определенных случаев, ССВЗК рекомендован к проведению всем пациентам с подтвержденным ПГА, планирующим оперативное лечение. На примере представленного клинического наблюдения пациентки с подтвержденной односторонней гиперпродукцией альдостерона, картиной неизмененного надпочечника по данным визуализирующих исследований и наличием гормонально - неактивного образования контралатерального надпочечника подчеркивается важность проведения ССВЗК для решения вопроса об оперативном лечении.

Background: In the Russian Federation, newborn screening comprises thyroid stimulating hormone determination to exclude primary congenital hypothyroidism. Screening is carried out throughout Russia. Neonatal TSH can be used to assess iodine deficiency and monitor iodine prevention programs.Objective: To assess and compare official statistical data on congenital hypothyroidism, the prevalence of hypothyroidism and iodine deficiency syndrome in children, as well as urinary iodine in the Russian regions.Materials and methods: The level of neonatal TSH was determined in 97.69% of children born in the Russian Federation in 2017. This article represents the results on the prevalence of hypothyroidism in the regions with various iodine availability. The correlation analysis was used to assess the relationship of CH incidence in newborns and iodine availability.Results: The calculated correlation coefficient, which was 0.2, reflects a weak relationship between the degree of iodine deficiency in the region and the number of newborns diagnosed with congenital hypothyroidism.Conclusions: In the Russian Federation, a law on universal salt iodization does not exist, and many regions are still in conditions of moderate or severe iodine deficiency. To assess the iodine status in these particular regions, we could use the results of newborn TSH screening

The steadily growing interest in studying of endocrine genetics is driven not only by the search for new pathologies but also by the most pressing need to develop methods for early diagnosis, treatment and management of patients. Though 20% of all the patients with von Hippel-Lindau syndrome do not have a family history of the disease; a genetic basis is a natural characteristic of the disease and determinates a phenotype. Despite the nonaggressive course, in general, a relative unpredictability of the syndrome signs onset and the lack of regular monitoring can increase the risk of surgery complications and cause a disability at a young age. The presented clinical case shows the need for a multidisciplinary approach to management of the patients with von Hippel-Lindau syndrome.

This study is due to the need for a detailed study of the time management functioning in the modern corporate governance system. Current works in the field of time management and all existing concepts are tied, first of all, to the individual person, but not to the whole corporation. Time management in corporate structures is reduced to individual employees or divisions. Therefore, in order to implement time management in the current corporate governance system, it is necessary to identify all its features. The purpose of the study is determined by the identified problem and is to study the features of time management implementation in the corporate governance system. The main general theoretical methods were used for the research: analysis, synthesis, deduction, comparison and correlation. A large volume of Russian and foreign sources in the theory of time management was analyzed, from which the most popular and promising works were selected. The main software products that can be used to simplify the implementation of time management in the corporate structure were considered. According to the results of the study, it became clear that the main specificity of corporate governance is the complexity of the organizational structure and difficulties in conducting control procedures regarding the work of not only the main divisions, but also subsidiaries.

Radioactive iodine, flying out of the destroyed reactor of the Chernobyl nuclear power plant, like a corona virus quickly spread throughout Europe. Iodine deficiency in the regions of Ukraine, Belarus and Russia adjacent to nuclear power plants became a factor in increased uptake of radioactive iodine by the thyroid gland in children and after 5 years led to an epidemic of thyroid cancer. Optimal iodine intake could become a kind of vaccination, which sharply reduces the risk of developing thyroid cancer, as has happened after the accident at the Fukushima nuclear power plant in Japan. Endemic goiter was eliminated 50 years ago, but returned to the country in the early 1990s after the collapse of iodized salt production and has not been eliminated to this day due to the lack of a legislative framework for mandatory salt iodization. The actual average consumption of iodine by residents of Russia is from 40 to 80 mcg per day, which is 23 times less than the recommended norm. Mild and moderate iodine deficiency was detected throughout the Russian Federation, and it is more typical for the the rural population. The iodine deficiency has the greatest negative effect on the psychomotor development of the child during the critical period the first 1000 days of life from the moment of conception to the end of the second year of life. According to WHO, over the past 20 years, iodine deficiency has been eliminated in 115 countries of the world, and the number of iodine-deficient countries has dropped to 25, but Russia is still among them. We believe that after the COVID-19 pandemic, it will no longer be necessary to prove the need for effective support for the prevention of both infectious and non-infectious diseases, and the declared preventive direction of Russian medicine will indeed become such.Радиоактивный йод, вылетевший из разрушенного реактора Чернобыльской атомной электростанции, как коронавирус, быстро распространился по Европе. Дефицит йода в прилегавших к атомной электростанции областях Украины, Беларуси и России стал фактором повышенного захвата радиоактивного йода щитовидной железой у детей и через 5 лет привел у них к эпидемии рака щитовидной железы. Оптимальное потребление йода могло бы стать своеобразной вакцинацией, резко снижающей риск развития поражения щитовидной железы, как это случилось после аварии на атомной электростанции в Фукусиме (Япония). Эндемический зоб устранен 50 лет тому назад, но вернулся в нашу страну в начале 1990-х годов после прекращения выпуска йодированной соли и остается не устраненным до настоящего времени из-за отсутствия законодательной базы для обязательного йодирования соли. Фактическое среднее потребление йода жителями России составляет от 40 до 80 мкг в день, что в 23 раза меньше рекомендованной нормы. На всей территории Российской Федерации выявлен йодный дефицит легкой и средней тяжести, причем в большей мере он характерен для питания сельского населения. Наибольший негативный эффект на психомоторное развитие ребенка йодный дефицит оказывает в критический период первые 1000 дней жизни с момента зачатия до конца второго года жизни. По данным Всемирной организации здравоохранения, за последние 20 лет йодный дефицит устранен в 115 государствах мира, а количество йододефицитных стран снизилось до 25, но Россия по-прежнему входит в их число. Полагаем, что после пандемии COVID-19 не надо будет доказывать необходимость эффективной поддержки в России мероприятий по предупреждению как инфекционных, так и неинфекционных заболеваний, а декларируемое профилактическое направление отечественной медицины действительно станет таковым.

Gravess disease is a common part of Autoimmune polyglandular syndrome (APS) and among thyroid autoimmune disorders is usually preceded the onset of the syndrome.The aim of this study was to determine the frequency of occurrence of APS type 2, 3 among patients with Graves disease.Sera of 94 patients with Gravess disease, 116 patients with APS 24 types and 80 healthy subjects were screened for 21-OH Ab, insulin-Ab (IAA), Islet Cell-Ab (ICA), glutamic acid decarboxylase-Ab (GADA), protein tyrosine phosphatase-Ab (IA2), Zinc Transporter 8-Ab (ZnT8), Anti-gliadin-Ab (IgA+IgG) (AGA), Anti-transglutaminase-Ab (IgA+IgG) (Anti-tTG), Anti-parietal cell-Ab (APCA), Intrinsic Factor-Ab (IF), Rheumatoid factor (RF), Anti Ovarian Antibodies (AOA). Serum cortisol, fasting plasma glucose levels were measured.The presence of Addisons disease and the onset of Type 1 DM was not determined among Graves disease patients. None of the patients with Graves disease and in the healthy control group had 21-OH-antibodies detected. The frequency of 21-OH-Ab was 4.2% in APS type 3 (p=0.07) and 91.6% in APS type 2, 4 (p0.001). The prevalence of diabetes-associated autoantibodies was 20.2% among Gravess disease patients against 8.75% in healthy subjects control group (p0.05); OR 2.64; 95% CI 1.056.66 and 30.2% in APS of adults (DM 1 negative group) (p=0.18). The prevalence of APCA-markers of autoimmune gastritis was 31.9% in Gravess disease, 48.3% in APS 24 types (p=0.01); OR 1.99; 95% CI 1.183.51, and 12.5% in control group (p0.01); OR 3.28; 95% CI 1.497.24. There were no significant differences in the frequency of occurrence of IF-Ab and RF-Ab in the groups. The frequency of AGA and ATA was 28.7% in Graves disease, 36.2% in APS types 24 (p=0.3), 10% in the control group ((р0.01); OR 3.63; 95% CI 1.548.54. Graves disease patients with risk of developing APS type 3 (positive diabetes-associated and other autoantibodies) had relatives with autoimmune diseases in 57.5% of cases (p=0.05); OR 2.18; 95% CI 1.034.63.Graves disease patients are at high risk for future development of APS 3 type, especially those with inheritance for autoimmune diseases. Screening for the immunological markers, pathognomonic for coexisting autoimmune diseases in such patients with Graves disease, as well as in patients with APS type 3, should be done regularly.Болезнь Грейвса (БГ) является часто встречающимся заболеванием в составе аутоиммунного полигландулярного синдрома (АПС) взрослых и среди аутоиммунных расстройств щитовидной железы обычно предшествует началу синдрома. Цель.Оценить распространенность латентных форм АПС 2, 3-го типов у пациентов с одним аутоиммунным заболеванием БГ в славянской популяции путем определения частоты встречаемости антител к другим органам-мишеням, компонентам АПС. Материалы и методы.В ходе исследования по типу случайконтроль исследованы образцы крови 94 пациентов с БГ, 116 пациентов с АПС 24-го типов, 80 здоровых добровольцев из группы контроля на предмет наличия иммунологических маркеров антител (Ат) к 21-гидроксилазе (21-ОН), IAA, ICA, GAD, IA2, ZnT8, AGA, Anti-tTG, PCA, IF, ревматоидному фактору (РФ), антиовариальных антител (АОА) у женщин, исследование уровня кортизола крови (утро), уровня глюкозы плазмы крови натощак. Результаты.Манифестация хронической надпочечниковой недостаточности и сахарного диабета 1-го типа (СД 1) у пациентов с БГ не выявлена. Ат к 21-ОН в группе пациентов с БГ и в группе контроля не обнаружены. В группе пациентов с АПС 3-го типа частота встречаемости Ат к 21-ОН составляет 4,2% (p=0,07), у пациентов с АПС 2, 4-го типов 91,6% (p0,001). Антитела к СД 1 (IAA, ICA, GAD, IA2, ZnT8) у пациентов с БГ выявлены в 20,2% случаев, что достоверно выше, чем в группе контроля, 8,75% случаев (p0,05); отношение шансов (ОШ) 2,64; 95% доверительный интервал (ДИ) 1,056,66, что не отличается от показателей в группе пациентов с АПС взрослых без СД 1 30,2% случаев (p=0,18). Частота встречаемости Ат к пернициозной анемии и аутоиммунному гастриту (PCA) у пациентов с БГ составляет 31,9%. Уровень Ат к PCA при БГ ниже, чем в группе пациентов с АПС взрослых, 48,3% (р=0,01); ОШ 1,99; 95% ДИ 1,183,51, однако достоверно выше, чем в группе контроля, 12,5% случаев (р0,01); ОШ 3,28; 95% ДИ 1,497,24. Достоверных различий в частоте встречаемости антител к IF и РФ в группах не выявлено. AGA (иммуноглобулин A + иммуноглобулин G) и Анти-tTG (иммуноглобулин A + иммуноглобулин G) у пациентов с БГ выявлены в 28,7% случаев, у пациентов с АПС взрослых в 36,2% случаев (p=0,3), в группе контроля в 10% случаев (р0,01); ОШ 3,63; 95% ДИ 1,548,54. У пациентов с БГ с положительным титром антител к ранее не диагностированным аутоиммунным заболеваниям наследственность отягощена в 57,5% случаев (p=0,05); ОШ 2,18; 95% ДИ 1,034,63. Заключение.Пациенты с БГ находятся в группе высокого риска развития АПС 3-го типа, в особенности имеющие отягощенную наследственность по аутоиммунным заболеваниям. Целесообразно регулярное проведение иммунологического скрининга на предмет новых ранее не диагностированных аутоиммунных заболеваний у пациентов из групп риска.

This article provides statistics of small business’ share in the Russian economy and its role in the development of the country’s innovative potential. The author concluded the presence of barriers that prevent the normal functioning of small business in the country, as well as in the Samara region. The main factors that slow down the development and effective functioning of small business and all entrepreneurship in Russia are highlighted. Within the framework of this topic, an analysis of the small business structure in the Samara region and a survey of individuals associated with small business were conducted. Based on this analysis, the author identifies a list of problems that stop the optimal functioning of small business. In addition, a significant result of this scientific work is the systematization of the control crisis and entrepreneurship support.

Nowadays, the choice of the right strategy for the development of the organization and ways that can increase the competitiveness plays a huge role in strengthening position of the company in the market. It is very important that the chosen development path corresponds to current trends under conditions of the economy digitalization and informatization of services used in the management and operation of enterprises. The authors analyze opinions of students and graduates of universities in the Samara region in order to identify their attitude to digitalization and the main factors affecting the competitiveness of enterprises in these digital environment. The research was carried out using survey methods, in-depth interviews, mathematical methods and specialized software products. The purpose of this study is to determine the impact of digitalization on the efficiency and effectiveness of methods to improve the competitiveness of modern enterprises and identify the attitude of young representatives of our society to this issue. To achieve this goal, the author set the following research tasks: to study the attitude of students and graduates of HEIs in the Samara Region to the influence of informatization on the competitiveness of companies; to determine the main factors that may affect the efficiency of organizations in modern conditions; to find out specific features of the change process in Russian companies in the conditions of the economy digitalization.

Nonalcoholic fatty liver disease (NAFLD) – a common chronic disease of the liver, the main feature of which is the accumulation of fat in hepatocytes, is not related to alcohol abuse. NAFLD is associated with obesity, especially abdominal, metabolic syndrome and various metabolic markers of risk for cardiovascular disease and type 2 diabetes, which affects the incidence, prognosis and life expectancy of patients with obesity. This lecture describes the main causes of the disease, its pathogenesis, clinical presentation, diagnosis and treatment.

Obesity is a chronic disease associated with cardiometabolic risk factors, primarily cardiovascular diseases and type 2 diabetes mellitus. In 2016,Russiaregistered a new drug for treating obese patients liraglutide 3.0 mg, which is an analogue of human glucagon-like peptide-1. Given the urgency of the problem, we present a clinical case that shows the efficacy of therapy with liraglutide 3.0 mg daily in a patient with obesity, and its effect on metabolic risk factors.

Hypoglycemic syndrome is a symptom complex that results from low blood sugar levels. In the endocrinologist practice, an insulinoma that is a small tumor in the pancreas that produces an excess amount of insulin is regarded as the main cause of hypoglycemia in patients without diabetes mellitus. Various molecular and genetic disorders develop in the insulinoma tissue, that lead to a change in the secretion of insulin and its precursors. There is often a situation when it is not possible to establish the cause of lowering blood glucose levels. In such cases, the development of hypoglycemia can result from various genetically determined enzyme, autoimmune and receptor disorders that cause a change in glucose metabolism or the synthesis/bioactivity of insulin. In the mild course of such congenital diseases, hypoglycemic conditions may first manifest in adulthood. The review describes various genetic predictors (mutations) that play a decisive role in the development of enzyme, autoimmune, receptor and proliferative disorders and, as a consequence, hypoglycemia.

Among comorbidities of obesity, hypothyroidism is the most common endocrinopathy. The pathogenesis of both diseases is based on serotonin deficiency. The article tells about a clinical case of a patient with obesity and subclinical hypothyroidism; a comprehensive examination allowed to choose the efficient and safe pathogenetic therapy.

Obesity is a chronic disease associated with cardiometabolic risk factors of cardiovascular diseases and type 2 diabetes. In 2016, in Russia a new drug was registered for the treatment of patients with adiposity - liraglutide 3.0 mg, which is an analog of human glucagonoma peptide-1. The study purpose: to study the efficacy of liraglutide 3.0 mg/day and its impact on cardiometabolic risk factors in patients with obesity. We examined 30 patients aged 25-59 with obesity without diabetes. Patients received therapy with liraglutide 3.0 mg/day for 3 months. The examination included measurement of anthropometric parameters, blood sampling with the estimation of the parameters of lipid and carbohydrate metabolism.Results: after 3 months of treatment with liraglutide 3.0 mg a significant improvement of anthropometric parameters was observed: reduction of weight, BMI and waist circumference and a significant improvement in parameters of metabolic comorbidities.Conclusions: complex therapy with use of liraglutide 3.0 mg per day for 3 months is effective for therapy of obesity and correction of obesity-associated cardiometabolic disturbances.

Backgraund: Obesity and type 2 diabetes mellitus (T2DM) are associated with with an increased risk of cardiovascular disease (CVD) and coronary artery disease (CAD), in particular. Obesity lead to several fibrotic processes, including activation of transforming growth factor (TGF-). Recent data indicate the involvement of Fibroblast growth factor 21 (FGF-21) as an important metabolic regulator, and even biomarker of metabolic changes in obesity and T2DM. Impact of metabolic dysregulation that accompany obesity and T2DM in CAD development remain a great challenge. Aims: To study TGF- and FGF-21 level in patients with obesity and T2DM. Materials and methods: TGF- and FGF-21 were identified in peripheral blood samples of 66 patients with obesity, aged 48-65 years. 1st group included 21 patients with CHD and T2DM; 2nd group (22 patients)- with T2DM and excluded CHD; 3rd group (20 patients) with normal glucose metabolism and excluded CHD. Results: TGF- was lower in patients with CHD (group 1) than in the group of "metabolically healthy" obesity (p=0.022). TGF- in patients with T2DM negatively correlated with LDL cholesterol (r=-0.426, p=0.038) the degree of internal carotid artery stenosis (r=-0.426, p=0.024). Patients with verified CHD had a negative correlation with the processes of heart muscle remodeling (thickness of the left ventricular posterior wall (r=- 0.386, p=0.029) interventricular septum (r=-0.335, p=0.031). All patients with obesity had significantly increased level of FGF-21 compared with the control group (p=0.031) FGF-21 positively correlated with BMI (r=0.473, p=0.033) Conclusions: TGF- has negative correlations with the factors that can influence prognosis and the severity of the CVD/. There were found correlations of FGF-21, TGF- with pathological angiogenesis and changes in normal cardiac geometry in obesity, T2DM and CAD.

The article is aimed at analyzing goals and objectives of staff motivation in the context of globalization of information processes, as well as some ways of providing material and non-material motivation of the staff taking into account actual trends formed in the modern world. The authors identified basic technological systems used in modern organizations to improve competitiveness and new ways of material and non-material motivation of personnel. Within the framework of this topic, the analysis of opinions was carried out and the correlation of different motivational approaches in the conditions of digitalization was revealed. In addition, a significant result of this research is the systematization of approaches to staff motivation in the context of the economy digitalization, taking into account the existing fundamental theories of motivation.

In the conditions of the digital transformation of the economy, the demand for highly qualified intellectual potential of employees increases, which objectively requires the integration of educational systems of various countries, the creation of strategic alliances in the field of higher and additional education, educational and business organizations. The rapid development of information technology and technological breakthroughs contribute to the development of innovation in every way. The purpose of this contribution is a structural and functional analysis of requirements to managers of innovative companies in the conditions of the digital economy.

This article provides our own clinical observation of the patient with multiple endocrine neoplasia type 2B (MEN2B) associated with a germinal mutation in the RET proto-oncogene. Although there are highly informative laboratory methods available, in clinical practice MEN2B syndrome is often diagnosed lately, which results in decrease in patients’ life expectancy and quality.

Hypertension is a very common disease with high morbidity and reduction in quality of life. Endocrine disorders are the most common cause of secondary hypertension affecting ~3% of the population. Primary aldosteronism can be the cause of endocrine hypertension more often than other endocrine disorders. Other less common causes of endocrine hypertension include Cushing syndrome, pheochromocytoma, thyroid disorders, and hyperparathyroidism. Endocrine hypertension is potentially curable if the underlying cause is identified and treated accordingly. Younger age at manifestation of resistance to multiple antihypertensive drugs, together with other clinical signs of an endocrine disorder, should raise the suspicion and prompt the appropriate evaluation.

In the article there are presented main approaches to the diagnosis, treatment and follow-up of patients with classical and nonclassical forms of 21-hydroxylase deficiency. The techniques of diagnostic tests and parameters of hormonal indices for the assessment of the adequacy of replacement therapy with glucocorticoids and mineralocorticoids are considered.

The relevance of this contribution is caused by the fact that following the growing need of development of small business in Russia, the organizations, representing small business, more often stop their functioning, than appear in the market as new players. According to the authors, an important challenge is the fact that the barriers, hindering development of small business, arise because of the developed negative attitude of the Russian society to a figure of an entrepreneur and business activity in general. Entrepreneurship is a phenomenon, which is not free from morals and values: The propensity to create serious business for the long-term perspective requires a set of value orientations, which determine what should be made and how it should be made. The purpose of this study is consideration of the existing approaches to understanding of the phenomenon of entrepreneurship from the point of view of value orientations, which are the basis for business activity in conditions of innovative development of the global economy. The results of the conducted research on the relevant values are presented in the work. The role of such valuable entrepreneurial quality as readiness to changes, necessary for formation of a new mode of thinking of the modern business community, is considered in this contribution. By results of the conducted research, the authors offered a model of this integrative characteristic, which can be the basis for purposeful process of preparation and retraining of entrepreneurs (including future ones) for development of the entrepreneurial culture of the region in the context of requirements of the innovative economy.

Summary: Natural iodine deficiency affects around 2 billion people worldwide. It is known that insufficient intake of iodine is especially dangerous at the stage of fetal development and early childhood. Changes caused by iodine deficiency in these periods of life bring about irreversible defects in the intellectual and physical development of children. A dramatic outcome of chronic iodine deficiency during fetal development and early childhood is mental disability. The urgency and procedure for iodine prophylaxis in pregnant and lactating women are defined by the World Health Organization (WHO) and the International Council for the Control of IDD (ICCIDD). Pregnant and lactating women are referred to the group of absolute risk for developing IDD. WHO recommends to administer potassium iodide to pregnant and breastfeeding women residing in the areas of iodine deficiency. The Order of the Russian Ministry of Health defined the list of essential drugs for women during pregnancy and the postpartum period which also includes potassium iodide medications. Russian guidelines for the level of iodine intake by pregnant and nursing women conform to the WHO recommendations. Federal standards of care for pregnant women stipulate prescription of potassium iodide medications.

Background . Previous surveys showed chronic iodine excess - median urinary iodine concentration (UIC) over 500 mcg/l — supposedly due to water contamination in population of Turinsk, a town in Sverdlovsk Region of Russia. Aim . Conduct an assessment of the effects of chronic iodine excess on thyroid function and spectrum of thyroid abnormalities in schoolchildren and adults. Materials and methods . Assessment was conducted in 100 schoolchildren and 100 adults residing in Turinsk and 97 schoolchildren and 100 adults in the control site (city of Pervouralsk) with optimal UIC. Assessment included thyroid ultrasonogrpahy, urinary iodine and dry spot TSH in schoolchildren and TSH and TPO-ab in adults. Iodine was also measured in random water samples from 10 households in Turinsk. Results . Median UIC in schoolchildren in Turinsk (719 mcg/l) was significantly higher (129 mcg/l) than in Pervouralsk resulting in higher prevalence of thyroid enlargement and TSH level. In adults TSH level as well as prevalence of sub-clinical hypothyroidism and autoimmune thyroiditis were higher in residents of Turinsk compared to control group from Pervouralsk. Conclusions . Iodine excess alters thyroid function in schoolchildren and adults increasing prevalence of thyroid enlargement in children and autoimmune thyroidis in adults.

Clinical practice recommendations are dedicated to the management of patients with Graves' disease and multinodular toxic goiter.

A critical consequence of chronic iodine deficiency during fetal development and early childhood is a violation of intellectual development. The need and the order of iodine prophylaxis in pregnant and lactating women identified the World Health Organization (WHO) and the International Council for Control of iodine deficiency disorders (IDD - ICCIDD). Pregnant and lactating women are referred to the absolute risk of developing IDD. WHO and ICCIDD recommend prescribe potassium iodide for women during pregnancy and breast-feeding, living in areas of iodine deficiency. Order № 50 of the Ministry of Health and Social Development of the Russian Federation from 2007 identified essential drugs for women during pregnancy and the postpartum period, which also made the preparations of potassium iodide. Russia's recommendations on the level of iodine intake by pregnant and nursing women conform to WHO recommendations. Federal standards of care for pregnant women include prescription drugs, potassium iodide.

The results of comparative analysis of the data on sensitivity and specificity of different methods for topical diagnostics of insulinomas are presented. It is concluded that MSCT and intraoperative ultrasound study are the most sensitive and specific methods for this purpose among the non-invasive and invasive techniques, respectively. An optimal algorithm for diagnostics of the tumours is proposed.

The present review is focused on epidemiology, clinical picture, and principal methods for laboratory and topical diagnostics of insulinomas. Results of the comparative analysis of Russian-language and foreign publications concerning sensitivity and specificity of various methods of topical diagnostics, postoperative complications and lethality from insulinomas are presented.

Type 1 diabetes mellitus is not infrequently associated with other autoimmune endocrine and non-endocrine diseases included in the notion of "autoimmune polyglandular syndrome" (APS). Their incidence in the patients presenting with this syndrome is significantly higher than that of isolated autoimmune pathologies. The enhanced risk of the development of these diseases is associated with the presence of genetic markers, such as HLAII-complex haplotypes, CTLA-4, PTPN22, FOXP2, and certain other genes. Decompensation of autoimmune thyroid disorders and adrenal insufficiency, as well as the presence of celiac disease, autoimmune gastritis and pernicious anemia in the patients with type 1 diabetes mellitus may be responsible for the impairment of metabolic control. It is recommended that the patients with DM1 be regularly examined for the early diagnostics of autoimmune diseases including components of APS and the associated subclinical functional disturbances with a view to preventing deterioration of metabolic control.

A total of 55 patients at the age from 18 to 60 years presenting with the classical forms of congenital adrenal hyperplasia (CAH) were available for the estimation of the body mass index (BMI), measurement of AP, glycemia in OGTT, insulin level, HOMA index, and lipid content in blood sera. BMI ≥ 25 kg/m2 was documented in 56% of the women and in 47% of the men with CAH. Arterial hypertension occurred in 10% and 14% of the women and men respectively, disturbances of carbohydrate metabolism were diagnosed in 17% of the women and 7% of the men, but men alone suffered diabetes mellitus. 35% and 47% of the women and men presented with insulin resistance respectively. Dyslipidemia concomitant with CAH occurred in 69% of the women and 50% of the men. Disturbances of carbohydrate metabolism and arterial hypertension were largely associated with the elevated BMI while dyslipidemia in the first place correlated with the degree of CAH compensation. It is concluded that the patients with CAH are subject to the high prevalence of metabolic disorders. Special attention should be given to the patients with an excessive body mass and those having episodes of iatrogenic hypercorticism in their medical histories. Such patients most frequently exhibit a combination of metabolic disorders that constitutes a risk factor of the development of cardiovascular complications.

The aim of the study was to compare clinical, laboratory and morphological parameters in patients with abdominal obesity and non-alcoholic fatty liver disease (NAFLD), and assessing the relationship between the degree of severity, stage NAFLD and cardiometabolic risk factors for type 2 diabetes mellitus and cardiovascular disease. The present study examined the content of adiponectin and rates of glucose and lipid metabolism in obese patients at different stages of NAFLD. According to the study, morphologically NAFLD was confirmed in 95.2% of patients. NAFLD was associated with various cardiometabolic disorders (dyslipidemia, disorders of glucose metabolism and insulin resistance), growing in frequency and severity with the progression NAFLD; and low levels of adiponectin decreasing with a deterioration NAFLD.

Biliopancreatic diversion (BPD) surgery is one of the most efficacious bariatric procedures employed to manage morbid obesity. However, apart from a stable reduction of the body weight and beneficial action on the clinical course of various diseases associated with obesity, this method creates the risk of inducing osteometabolic disorders following the surgical intervention. We present here a clinical case of osteomalacia that developed in the late post-BPD period in the absence of regular intake of medicines and adequate medical care.

Von Hippel-Lindau disease is a hereditary tumour syndrome associated with the earlier development of a variety of benign and malignant neoplasms, such as hemangioblastomas of the central nervous system and retina, tumours of the internal ear, renal carcinoma and cysts, pheochromocytoma, neuroendocrine tumours, pancreatic cysts, epididymal and broad ligament cystadenomas in men and women respectively. Von Hippel-Lindau disease is considered to be the most common cause of hereditary renal cancer.

Представленные клинические рекомендации объединяют мнения членов рабочей группы по ключевым и наиболее спорным проблемам диагностики и лечения МРЩЖ, которые сложились в отечественной клинической практике. В рабочую группу вошли специалисты, работающие в различных лечебных и научно-исследовательских учреждениях.Этот документ не является официально утвержденным различными структурами Министерства здравоохранения и социального развития РФ. Представленные в нем положения носят рекомендательный характер.Основные предпосылки

The congenital form of pheochromocytoma is known to be fraught with high risk of post-treatment relapse, bilateral, multicentric or primarily multiple lesions. The patients presenting with the syndrome of multiple type 2 endocrine neoplasia usually have no extra-adrenal pheochromocytomas or metastases. Both examination and treatment of these patients should be performed taking these peculiarities into consideration. We describe a family with multiple type 2 endocrine neoplasia and highlight selected aspects of the management of the patients presenting with genetically determined pheochromocytoma.

The present work was designed to study parameters of calcium and vitamin D metabolism in the patients presenting with morbid obesity (MO) including those who had undergone biliopancreatic diversion bypass (BPD). The patients were allocated to three groups. Group 1 was comprised of the patients with BMI in excess of 40 kg/sq.m. (n=22), group 2 included the patients after BPD (n=23), and group 3 (control) consisted of the healthy volunteers having normal body weight in the absence of signs of obesity (n=22). The three groups were significantly different in terms of consumption of vitamin D (p

Non-alcoholic fatty liver disease (NAFLD) is a common chronic disease, uniting clinical and pathological changes in a liver: steatosis, non-alcoholic steatohepatitis, fibrosis and cirrhosis. NAFLD is associated with obesity especially of abdominal type and metabolic syndrome, that raises cardiometabolic risk and effects morbidity, prognosis of the disease and life expectancy of patients. This article describes the basic mechanisms of development of NAFLD, classification, prognosis of the disease, diagnosis and treatment.

Rehabilitation of patients with severe traumatic brain injury (sTBI) is a topical medical and social issue because this pathology is one of the main causes of mortality and disability in the young working age population [1]. The most common sTBI consequences include motor and cognitive impairment as well as depression of consciousness [2, 3]. Despite significant progress in treatment of the consequences of severe traumatic brain injury, there are no treatment and rehabilitation standards for these patients, and the used rehabilitation measures are not always effective. These circumstances substantiate the need for the development of additional methods of neurotherapy. Over the past decade, transcranial electrical and magnetic stimulation (TMS) has been increasingly used as neuromodulatory treatment in clinical practice [4-12]. The accumulated experience has shown that transcranial neurostimulation methods require a more individualized approach in terms of both careful selection of patients and choice of exposure parameters. This review is based on an analysis of the most significant publications and recommendations recognized in the scientific community, as well as on reports of domestic and foreign authors presented at dedicated congresses in comparison with experience of our own research on transcranial stimulation. The paper discusses the main problems of using this method in medical practice of sTMI and their possible solutions.Реабилитация пациентов с тяжелой черепно-мозговой травмой (ТЧМТ) является актуальной медико-социальной проблемой, поскольку эта патология остается одной из основных причин летальности и инвалидизации населения молодого трудоспособного возраста. К числу наиболее распространенных последствий ТЧМТ относятся двигательные и когнитивные нарушения, а также угнетение сознания. Несмотря на значительные успехи в терапии последствий тяжелого повреждения мозга, лечебно-реабилитационные стандарты для таких пациентов на сегодняшний день отсутствуют, а применяемые реабилитационные мероприятия не всегда эффективны. Эти обстоятельства обосновывают необходимость разработки дополнительных методов нейротерапии. В последнее десятилетие в клинической практике в качестве нейромодуляторного воздействия все шире применяется транскраниальная электрическая и магнитная стимуляция (ТМС). Накопленный опыт показал, что методы транскраниальной нейростимуляции требуют более индивидуализированного подхода, касающегося как тщательного отбора пациентов, так и выбора характеристик воздействия. Настоящий обзор основан на анализе наиболее важных и признанных в научном сообществе публикаций и рекомендаций, а также докладов отечественных и зарубежных авторов, представленных на тематических конгрессах, в сопоставлении с опытом собственных исследований по транскраниальной стимуляции. Обсуждаются основные проблемы использования этого метода в процессе ведения пациентов с ТЧМТ и возможные пути их решения.