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Hippel-Lindau disease is a rare hereditary combined pathology characterized by development of a variety of benign and malignant tumors in the central nervous system, kidneys, pancreas, adrenal glands, which causes a wide range of clinical manifestations. A comprehensive diagnostic approach using various imaging techniques such as ultrasound, magnetic resonance imaging, computed tomography, and genetic testing is an integral part of successful treatment.

Background: Numbers of patients with diabetes mellitus using insulin pumps have been increasing every year. Successful achievement of glycemic targets with continuous subcutaneous insulin infusion (CSII) is based on an adequate basal rate of infusion, carbohydrate coefficient and insulin sensitivity index. There are two approaches to basal insulin infusion rate, namely the flat one and the circadian; however, at present there is no convincing data on which one should be chosen at the start of insulin pump therapy.Aim: To compare two regimens of basal insulin infusion rate at initiation of insulin pump therapy in routine clinical practice.Materials and methods: We analyzed data from 120 patients with Type 1 diabetes mellitus, who were switched on insulin pump therapy in the Department of Endocrinology from 2017 to 2018. At initiation of CSII, 60 patients used the flat basal rate profile and the other 60 patients used the circadian basal rate, calculated with the Renner's scale. Safety of the two basal rate regimens was assessed based on glucose variability measured with continuous glucose monitoring during the first two days after the start of insulin pump therapy.Results: Mean (± SD) coefficients of variation in the groups with circadian and flat basal rate at Day 1 were 31.06±12.13 and 32.74±10.7, respectively (p=0.423); at Day 2, 26.78±11.27 and 28.83±10.7 (p=0.309). Median [Q1; Q3] areas under glucose curve (AUC) values above the glucose targets in the groups with circadian and flat basal rate at Day 1 were 0.37 [0.03; 0.89] and 0.48 [0.08; 1.75], respectively, at Day 2 0.44 [0.03; 1.57] and 0.31 [0.1; 1.5], respectively (p>0.05). Median glucose AUC values below the goal in groups with circadian basal rate and flat basal rate on the first day were 0.01 [0; 0.06] and 0.02 [0; 0.1], respectively (p=0.855), on the second day – 0.00 [0; 0.01] and 0.00 [0; 0.02], respectively (р=0.085). We also haven’t found any between-group differences in the prevalence of glucose deviations below and above the target, as well as in the time spent in normoglycaemia.Conclusion: The comparative analysis of two basal insulin rate regimens in Type 1 diabetic patients switched to insulin pump therapy has shown no significant differences between them. The use of Renner’s scale has no clinical advantages over the fixed basal insulin regimen at initiation of insulin pump therapy in adults.

Introduction. Vessel wall imaging in patients with dissection plays an impotent role in the differential diagnosis of stenoocclusive processes of the main arteries of the head. However, the interpretation of changes in long-term periods remains difficulties. Purpose: to determine and compare the imaging patterns of the consequences of the postponed dissection using MRI and ultrasound.Materials and methods. 30 patients with confirmed dissection were examined for more than 1 year from the date of diagnosis. MRI was performed using vessel wall protocol imaging before and after contrast enhancement.Results. Postpone dissection imaging patterns were revealed: aneurysmal dilation of the artery (27%) and double lumen (20%) at the site of dissection; prolonged stenosis in 10% of cases, intimal flap — in 3% of cases. Occlusion persisted in 40% of cases.Conclusion. The use of MRI can improve the differential diagnosis of the causes of the stenоocclusive process of the main arteries of the head.

Aim. To evaluate the effectiveness a new complex including aerobic and physical training, kinesohydrotherapy, balance therapy onchanges in muscle strength and correction of motor disorders in obese patients. Material and methods. The study group included 40 patients aged 58 [53;66] y.o. with a BMI≥30 kg/m2. The control group includedof 40 people aged 57 [54;63] y.o. with BMI≥30 kg/m2. Study methods included anthropometry, functional tests and dynamometry. Results. According to the data obtained, in both groups after treatment, body weight significantly decreased (from 106.03 [83;145] to102.8 [80;141] kg), BMI (from 39.2 [30.12;49.1] to 38.1 [29.4;46.7] kg/m2), decreased WС (from 109 [105;125.8] to 107 [98.8 12] cm), HС(from 127 [112.3;139.8] to 121 [109.5;133.5] cm), decreased pain syndrome (from 5 [3;7] to 2.5 [1;4.75] points), increased arm strength(in right arm from 20 [14.25;34] to 30 [19;42], in left arm from 19.5 [14.25;29.5] to 22 [18;30.75] daN). Conditioning and coordinationabilities improved significantly in the main group according to the functional tests: “Up and go test” (from 7.9 [7.1;8.9] to 7.4[6.5;8.3] sec.), back muscle strength (from 5[5;5] to 5[5;5]), static and dynamic abdominal muscle endurance (from 12.04 [9.47;17.13]to 16.07[10.69;27.7] sec. and from 31[21;37.25] to 39 [29.5;46.5] sec, and back and (from 14.94[5.8775;22.205] to 18.41[9.745;31.335]times and from 8[5;14] to 10 [8;23], times, respectively); Fukuda test scores (from 65[56;76.75] to 72[61;82] reps), One leg standing test(from 13.9[5.38;32.15] to 18.61[8.6125;38.1575] sec. for the left) and closed eyes (from 3.45[2.16;6.38] to 3.975[2.715;5.82] seconds forthe right and from 4.12[1.3;8.61] to 4.31[2.16;8.13] seconds for the left). Conclusions. A new complex including aerobic and strength training, kinesohydrotherapy, and balance therapy showed significanteffects on body weight reduction, body volume reduction, and muscle strength improvement in obese patients. A new integratedmethod results in a longer maintenance of the achieved effect when controlling the long-term results after 3 months and 1 year comparedwith the group that received only the 2-component program.

Dissection of the internal carotid (ICA) and vertebral arteries (VA) is one of the most common causes of ischaemic stroke in younger people. Despite wider use of modern neuroimaging techniques in clinical practice, and greater diagnostic capabilities for assessing structural changes in blood vessels, detection of spontaneous dissection remains low. The aim of the study was to identify patterns of change in the signal characteristics of an intramural haematoma in patients with spontaneous ICA and VA dissection on MRI and MR angiography (MRA), during the first three months after onset of clinical symptoms, as well as to create an optimal scan protocol. Based on changes in the K value and a visual assessment of the signal characteristics of the intramural haematoma, the study found that the signal intensity from the haematoma depends on the time of the examination. In the acute and chronic stages of dissection, the K value approaches 1 under Т1 FS and time-of-flight (TOF) MRA modes, and the intramural hematoma “merges” with the surrounding soft tissues, making it difficult to detect during these periods. Between 7 days and 2 months, the haematoma has a hyperintense signal and the K value is >1 under Т1 FS and TOF-MRA modes (p < 0.05). A comparison of K value data from different time periods made us add the T1 FS mode to the scan protocol for patients with suspected spontaneous dissection, in addition to the standard TOF-MRA, in order to improve the intramural haematoma imaging.

Time course of psychological status of diabetics during automonitoring training is analyzed. Seventeen patients with insulin-dependent diabetes mellitus aged 15-43 years with disease standing of 1-17 years were followed up, who were never trained automonitoring before. Two control groups were not trained, which helped assess the results from different viewpoints. The levels of depression, anxiety, energy, positive sensations, total well-being, satisfaction with treatment, subjective perception of hypoglycemic reactions, and subjective perception of hyperglycaemia were assessed using special questionnaires. Compensation of diabetes was improved both in the main and control groups. Psychological status of diabetics improved after training: levels of depression and anxiety decreased, levels of energy, psychological well-being, and general psychological status increased (p

BACKGROUND: Data of real clinical practice in diabetes mellitus (DM) register allow to evaluate features and trends in structure of glucose-lowering therapy (GLT). AIM: Тo analyze of structure of GLT received by patients with type 2 diabetes mellitus (T2DM) in Moscow region for 2018 and to evaluate its dynamics over 15 years. METHODS: Analysis of GLT structure was carried out on basis of data from register of patients with DM in Moscow region, which is part of National register of diabetes mellitus in Russian Federation. In March 2018 it contained data on 211,792 T2DM patients of Moscow region. Structure of GLT administration was evaluated according T2DM duration, patients age and presence of cardiovascular diseases (CVD). Dynamics of GLT is analyzed from 2004 to 2018 yrs. RESULTS: In 2018 non-insulin glucose-lowering drugs (NIGD) prescription prevailed (78.3%), insulin therapy was prescribed in 18.5% of patients, 3.2% of patients did not receive drug therapy. Most commonly prescribed NIGD were metformin (69.3%) and sulfonylurea (51.3%). Older patients more often than younger did not use GLT at all and less frequently received insulin therapy and iDPP-4. Insulin therapy was prescribed twice as often in patients with CVD compared with patients without CVD (29.6% and 15.5%). NIGD monotherapy has been less commonly used in patients with CVD (67.3% and 81.2%). Glucagon-like peptide-1 receptor agonists (GLP-1 RA) were prescribed to patients with CVD GLP-1 RA in 0.1% of cases, without CVD in 0.3% of cases, and sodium-glucose cotransporter 2 (SGLT2) inhibitors in 1.1% and 0.6%. correspondently. CONCLUSION: Metformin was most commonly prescribed drug in GLT structure for T2DM patients in the Moscow region in 2018 yr. Percentage of new drugs in the structure of GLT increased mainly due to iDPP-4, and secondly due to SGLT2 inhibitors. New classes of GLT were more often prescribed to patients of younger age, with diabetes duration up to 10 years, overweight or obese. Administration of NIGD with proven cardiovascular protection in presence of CVD is almost two times less than for those without CVD.

Background: Injectable somatostatin receptor ligands (iSRLs) have been a mainstay in acromegaly treatment. Oral octreotide capsules (OOC; MYCAPSSA®) were recently approved in the United States. Results from the placebo-controlled CHIASMA OPTIMAL and open-label CH-ACM-01 studies showed an OOC safety profile consistent with that of iSRLs with no new or unexpected safety signals. Results of the MPOWERED trial have enabled a comparison of OOC safety and efficacy with iSRLs. Methods: To enter MPOWERED, patients must have the following: acromegaly diagnosis, biochemical control of acromegaly (insulin-like growth factor I Results: In the RCT, incidence of treatment-emergent adverse events (TEAEs) was similar between groups; 39 patients (70.9%) in the OOC group and 26 (70.3%) in the iSRL group had ≥1 TEAE. 19 patients (34.5%) in the OOC and 15 (40.5%) in the iSRL group had treatment-related TEAEs. Occurrence was similar for serious AEs (OOC, 5.5%; iSRL, 8.1%) as well as TEAEs classified as severe (OOC, 9.1%; iSRL, 10.8%). One patient in the OOC group discontinued due to a TEAE. The most common gastrointestinal TEAEs were flatulence (OOC, 25.5%; iSRL, 21.6%), nausea (OOC, 20.0%; iSRL, 8.1%), diarrhea (OOC, 10.9%; iSRL, 13.5%), abdominal pain (OOC, 9.1%; iSRL, 8.1%), and constipation (OOC, 5.5%; iSRL, 13.5%). AEs of interest were infrequent, including cholelithiasis (OOC, n=0; iSRL, n=1 [2.7%]) and secondary hypothyroidism (OOC, n=1 [1.8%]; iSRL, n=0). In the iSRL group, 32.4% of patients reported injection site reactions (ISRs) during the RCT, and 47% of patients reported ISRs as part of the Acromegaly Treatment Satisfaction Questionnaire, a newly validated patient-reported outcome tool.1 Conclusion: Safety results from MPOWERED align with prior trials, showing that the OOC safety profile is consistent with that of iSRLs as well as the acromegaly disease burden. No new or unexpected safety signals were identified during the trial. Safety results were mostly similar between OOC and iSRLs, although patients in the OOC group did not experience any ISRs. 1Fleseriu M, et al. Pituitary. 2020 Aug;23(4):347-358.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system, mainly affecting the brain stem, cerebellum and spinal cord. The clinical picture includes gradually developing ataxia, double vision, dysarthria, pyramidal and cognitive impairment. Morphological examination reveals T-cell perivascular lymphocytic infiltration with CD4 lymphocytes predominance over CD8 lymphocytes. The cause of the disease is unknown. The article describes two patients (a 18-year-old woman and a 40-year-old man) with typical clinical and MRI manifestations of CLIPPERS, which was confirmed by brain biopsy in the female patient. The duration of follow-up was 3 and 7 years, respectively. Both patients survived an infection 2-3 weeks before the onset of disease that allows one to discuss its role in CLIPPERS pathogenesis. Both patients had a clear clinical and MRI responsiveness to steroids. In the female patient, steroids were replaced by intramuscular administration of the TNF-α blocker adalimumab. During 1,5 years of its use, there were no clinical relapses and pathological brain changes on MRI.Хроническое лимфоцитарное воспаление с периваскулярным накоплением контрастного вещества в мосту мозга при магнитно-резонансной томографии (МРТ) головного мозга и хорошим эффектом от лечения стероидами (

Aim. Assessment of prevalence and risk factors of sleep breathing disorders in patients with acromegaly from Moscow region. Materials and methods. Cardiorespiratory monitoring was executed to 55 patients with acromegaly (18 men and 37 women): 27 patients with de novo disease, 28 patients on treatment of acromegaly (including 18 patietns with uncontrolled and 10 - with controlled acromegaly). All subgroups did not differ on sex, BMI and age. Also 24-hour monitoring of arterial blood pressure was carried out in 39 patients (12 men and 27 women, 14 patients with de novo acromegaly, 15 and 10 patients with uncontrolled and controlled acromegaly, respectively). Results and discussion. The high prevalence of sleep breathing disorders (SBD) was revealed in patients with acromegaly from Moscow Region. SBD was found in 92.6%, 83.5% and 70.0% patients with newly diagnosed, uncontrolled and controlled acromegaly, respectively. The majority of patients had severe/moderate SBD in all subgroups (78.8%, 72.2% and 60.0%, respectively). In patients with newly diagnosed and uncontrolled acromegaly index of apnea-hypopnea (31 and 38.5 respectively), number of apnoe episodes (76 and 72) and saturation level (93% and 93.5%) did not differ significantly while these parameters were better in patients with a controlled acromegaly (apnea-hypopnea index 20, apnea episodes 45.5 and saturation level 95%). The peak of desaturation was subphysiological in 91.7%, 86.7% and 77.8% of patients with newly diagnosed, uncontrolled and controlled acromegaly, respectively. Severity of SBD did not depend on GH and IGF-1 levels as well as acromegaly duration. Such all-population risk factors of SBD as BMI and age were valuable for patients with acromegaly, however gender did not matter. SBD were associated with lack of physiological decrease of systolic and diastolic night BP. Conclusion. Acromegaly per se is a strong risk factor of sleep breathing disorders. The high prevalence of sleep breathing disorders in patients with acromegaly even after achievement of control over a disease emphasized need of specialized treatment of these violations.

The aim of this paper is to construct a mathematical model that takes the main physiological parameters of blood-glucose regulation into account, in order to identify these parameters for an individual patient according to continuous glucose-monitoring data. The constructed mathematical model consists of six ordinary differential equations that describe the dynamics of changes in glucose concentrations, as well as insulin and anti-insulin factors in the blood. Estimation of the parameters of the equations was performed using an evolutionary programming method. The model predictions were fitted to the continuous glucosemonitoring data. As a result of the identification of the model parameters for two patients with type 1 diabetes mellitus, the estimated insulin secretion was close to zero and the estimated glucose utilization and insulin clearance were increased in comparison with the data for healthy donors. Here, we present a personalized model of the regulation of blood glucose, which can be used to predict the results of continuous glucose monitoring depending on modification of the prescribed glucose-lowering therapy. This approach can significantly reduce the number of iterations of the selection of medical hypoglycemic therapy and therefore increase the effectiveness of treatment according to glucose-monitoring data.

[English] A summary of the draft federal clinical guidelines on osteoporosis developed by members of the Russian Association of Endocrinologists, the Russian Association for Osteoporosis, the Association of Rheumatologists of Russia, the Association of Traumatologists and Orthopedists of Russia, the Russian Association for Menopause and the Russian Association of Gerontologists and Geriatrics is presented. The recommendations were developed from the perspective of evidence-based medicine, in accordance with the requirements for compiling clinical recommendations of the Ministry of Health of Russia published in 2019. A significant place is given to screening of primary osteoporosis in adults, differential diagnosis with other metabolic diseases of the skeleton, modern methods of diagnosing osteoporosis, principles of prescribing pathogenetic treatment, features of sequential and combination therapy, disease prevention and rehabilitation. Clinical recommendations will be useful both to general practitioners and physicians, as well as to narrow specialists, primarily endocrinologists, rheumatologists, orthopedic traumatologists, nephrologists, obstetrician-gynecologists and neurologists, since osteoporosis is a multifactorial and multidisciplinary disease. [Русский] Представлено краткое изложение проекта федеральных клинических рекомендаций по остеопорозу, разработанных членами Российской ассоциации эндокринологов, Российской ассоциации по остеопорозу, Ассоциации ревматологов России, Ассоциации травматологов-ортопедов России, Российской ассоциации по менопаузе и Российской ассоциации геронтологов и гериатров. Рекомендации разработаны с позиций доказательной медицины, в соответствии с требованиями к составлению клинических рекомендаций Минздрава России, опубликованными в 2019 г. Значительное место в изложении отведено скринингу первичного остеопороза у взрослых, дифференциальной диагностике с другими метаболическими заболеваниями скелета, современным методам диагностики остеопороза, принципам назначения патогенетического лечения, особенности последовательной и комбинированной терапии, профилактики заболевания и реабилитации. Клинические рекомендации полезны будут как врачам общей практики и терапевтам, так и узким специалистам, прежде всего эндокринологам, ревматологам, травматологам-ортопедам, нефрологам, акушерам-гинекологам и неврологам, так как остеопороз является мультифакториальным и мультидисциплинарным заболеванием.

The complicity of religion in the mobilization of the population is undeniable being, but the functions of this institution may differ, depending on the current regime, the specific situation, the crisis or the need for change. In connection with the formation and development of religious education, specific historical events reflecting the stabilizing / destabilizing functions of religion have been considered in this article. Also, the ratio of religious and secular education has been presented by the author. The forms of interaction between religion and education in the system of modern society have been listed. Some educational institutions that carry out religious education today have been indicated.

Cervical artery dissection is the common cause of ischemic stroke in young and middle-age patients. According to our previous studies, dissection is related to arterial wall dysplastic changes, which in their turn are due to mitochondrial cytopathy. The authors describe three male patients who at the age of 53, 25 and 35 years underwent internal artery (ICA) dissection with occlusion of its lumen and subsequent recanalization in one of them. In 3.5 months, 13.5 years and 3 years respectively, patients developed intracerebral hemorrhage (IСH), which was not related to arterial hypertension, cerebral arterial aneurysms and anticoagulants. IСH were located on the side of ICA occluded after dissection (2 patients) or bilaterally in the territory of patent ICA (1 patient). MultivoxelДиссекция артерий шеи - частая причина ишемического инсульта в молодом и среднем возрасте. Согласно нашим предыдущим исследованиям, она связана с диспластическими изменениями артериальной стенки, в развитии которой имеет значение митохондриальная цитопатия. В работе описаны 3 больных мужчин, которые в возрасте 53, 25 и 35 лет перенесли диссекцию внутренней сонной артерии (ВСА) с окклюзией ее просвета и последующей реканализацией у одного из них. Через 3,5 мес, 13,5 и 3 года соответственно у больных развилось внутримозговое кровоизлияние, которое не было связано с артериальной гипертонией, аневризмой артерий головного мозга или приемом антикоагулянтов. Кровоизлияния располагались на стороне окклюзированной после диссекции ВСА (2 больных) или билатерально в бассейне проходимых ВСА (1 больной). Мультивоксельная ¹H-МР-спектроскопия, проведенная 1 больному на 40-й и 48-й дни после развития кровоизлияния, выявила высокий пик лактата в области визуально не измененного белого вещества полушарий. Предполагается, что митохондриальная цитопатия у больных с диссекцией может вовлекать не только крупные, но и мелкие интрацеребральные артерии - митохондриальная микроангиопатия, которая и может быть причиной внутримозговых кровоизлияний.

Twenty-five patients with type 2 diabetes (DM-2) and its moderate initial decompensation (8.83±1.85%) were examined. The mean age of the examinees was 53.16±8.92 years; the duration of the disease was 2.50±3.06 years. There were 18 females (mean age 54.11 ±9.67 years) whose disease duration was 2.71±3.08 years and 7 males (mean age 50.7 1±6.63 years) with a disease duration of 1.95±3.17 years. Before prescribing metformin, all the patients received diet therapy, but without making a special monitoring of the calorie content of a diet. All the patients were given metformin in an initial daily dose of 500 mg. Later on the daily dose of metformin was increased up to 1000-1500 mg. Metformin treatment was performed during low-calorie diet (as high as 1200 kcal daily); compliance was monitored by an individual dietary diary. In patients with DM-2, metformin exerted a positive effect on fasting glycemia (p = 0.001) and postprandial hyperglycemia (absolute and relative areas under the curve in the oral glucose tolerance test (OGTT)). At the same time there were no perceptible changes in the level of glycated hemoglobin (p = 0.533). Although metformin caused a noticeable decrease from 5.75±4.72 to 3.97±2.71, it was yet statistically insignificant (p = 0.181). In patients with DM-2, metformin therapy enhanced a low-calorie diet-induced body weight loss, most markedly within the first three months of therapy. At the same time, a meta-analysis has shown that this effect of metformin is rather moderate (p = 0.02). Metformin treatment for MD-2 during low-calorie diet improves lipid metabolism, by significantly decreasing the levels of total cholesterol and low-density lipoproteins, and lowers systolic blood pressure.Обследовано 25 больных сахарным диабетом 2-го типа (СД2) с умеренной исходной декомпенсацией диабета (8,83 ± 1,85%). Средний возраст обследованных - 53,16 ± 8,92 года, стаж заболевания - 2,50 ± 3,06 года. Было 18 женщин (средний возраст 54,11 ±9,67 года) со стажем заболевания 2,71 ± 3,08 года и 7 мужчин (средний возраст 50,71 ± 6,63 года) со стажем диабета 1,95 ± 3,17 года. До назначения метформина все больные находились на диетотерапии, но без специального контроля за соблюдением калорийности диеты. Всем больным был назначен метформин в начальное дозе 500 мг в сутки. В дальнейшем доза метформина повышалась до 1000-1500 мг в сутки. Лечение метформином проводили на фоне субкалорийной диеты (ограничение до 1200 ккал в сутки), соблюдение которой контролировалось по индивидуальному дневнику режима питания. У больных СД2 метформин оказывает положительное влияние на гликемию натощак (р = 0,001) и параметры постпрандиальной гипергликемии (абсолютные и относительные площади под кривой в ПТТГ). В то же время отсутствовала заметная динамика уровня гликированного гемоглобина (р = 0,533). Хотя метформин и вызвал заметное снижение индекса инсулинорезистентности с 5,75 ± 4,72 до 3,97 ± 2,71, но оно оказалось статистически незначимым (р = 0,181). У больных СД2 лечение метформином усиливает вызываемое гипокалорийной диетой снижение массы тела, причем наиболее заметно в первые 3 мес лечения. Вместе с тем метаанализ показал, что этот эффект метформина является довольно умеренным (р=0,02). Лечение больных СД2 метформином на фоне гипокалорийной диеты сопровождается улучшением липидного обмена (достоверно снижаются уровни общего холестерина и липопротеидов низкой плотности) и снижением систолического артериального давления.

There are no large-scale epidemiological studies on primary hyperparathyroidism (PHPT) in Russia. The high prevalence of the disease, the high risk of disability and death in this cohort of patients requires the study of the epidemiological and clinical structure of PHPT to determine the extent of medical care.Evaluate the frequency of PHPT detection and characterize its clinical forms in Russia using an online registry.The object of the study is the database of the State Register of Patients with PHPT 1914 patients from 71 regions of the Russian Federation. New cases of the disease, as well as dynamic indicators are recorded when patients visit outpatient clinics or medical institutions. The analysis of data made at the end of December 2017 was carried out. The following parameters were evaluated: demographic and clinical indicators; indicators of phosphorus-calcium metabolism, the main forms of PHPT and its course, the primary characteristic of PHPT in hereditary syndromes and parathyroid carcinoma. Results are presented as mean and standard deviations, or medians and quartiles; descriptive statistics of qualitative attributes absolute and relative frequencies.the total number of patients with PHPT in the registry on 31 of December 2017 was 1914 cases (0.001% of the population of the Russian Federation). Identification of PHPT was 1.3 cases per 100 thousand of the population in Russia, 7.6 cases in Moscow, 6.1 cases per 100 thousand in the Moscow region. The average age of patients at the time of diagnosis was 55.6 10 years. The active phase of the disease was registered in 84.6% of patients (1620/1914), most of whom had a symptomatic PHPT 67.1% (1087/1620), and 32.9% a asymptomatic disease (533/1620). Symptomatic disease with visceral complications was detected in 15.8% cases (172/1087), with bone complications in 48.4% (526/1087). The mixed form of the disease was detected in 35.8% of patients with manifest form (389/1087). Normocalcemic variant PHPT (nPHPT) was registered in 14.5% cases (234/1620). Sporadic PHPT occurs in 83% of cases (1592/1914). 326 patients (17%) had a suspicion for hereditary form of the disease: average age was 31.2 12.3 years. A genetic analysis was conducted in 61 patients (3.2%): showed the mutation in the MEN1 gene in 2.9% of cases (55/1914) and the mutation in the CDC73 gene in 0.3% of cases (6/1914) (HPT-JT syndrome). Parathyroid carcinoma was confirmed in 1.8% of all patients (35/1914). Surgical treatment was performed in 64.5% of patients (1234/1914). Remission was achieved in 94% of cases (1160/1234), in 6% of cases relapse after surgical treatment or persistence of PHPT was recorded.detection of PHPT in the Russian Federation raised in comparison to 2016, which is associated with an active start of registration of patients in the regions. At this stage, it is necessary to modify the principles of registration and control, to make a platform for gathering information and calculating the necessary volumes of medical care for PHPT patients.Обоснование. В России отсутствуют широкомасштабные эпидемиологические исследования по первичному гиперпаратиреозу (ПГПТ). Широкая распространенность заболевания, а также повышение риска инвалидизации и смерти у данной когорты пациентов диктуют необходимость изучения эпидемиологической и клинической структуры ПГПТ для определения объема медицинской помощи. Цель: оценить распространенность ПГПТ и охарактеризовать его клинические формы в России с помощью онлайн регистра. Методы. Объектом исследования является база данных регистра пациентов с ПГПТ 1914 пациентов из 71 региона РФ. Проведен анализ данных, внесенных на конец декабря 2017 г. Оценены следующие параметры: демографические и клинические показатели, показатели фосфорно-кальциевого обмена, основные формы ПГПТ и его течение, представлена первичная характеристика ПГПТ в рамках наследственных синдромов и злокачественного поражения околощитовидных желез. Результаты представлены средними и среднеквадратичными отклонениями, либо медианами и квартилями, описательная статистика качественных признаков абсолютными и относительными частотами. Результаты. Общая численность пациентов с ПГПТ в регистре на 31.12.17 составила 1914 человек (0,001% населения РФ). Выявление ПГПТ на 100 тыс. населения составляет по Российской Федерации 1,3 случая, в Москве 7,6 случая, в Московской области 6,1 случая. Средний возраст пациентов на момент установки диагноза составил 55,610 лет. Активная фаза заболевания зарегистрирована у 84,6% (1620/1914) пациентов, большая часть которых имела манифестное течение ПГПТ 67,1% (1087/1620), а 32,9% (533/1620) мягкое. При манифестном течении смешанная форма заболевания выявлена у 35,8% (389/1087) пациентов; изолированная костная форма у 48,4% (526/1087), изолированная висцеральная у 15,8% (172/1087). Нормокальциемический вариант ПГПТ (нПГПТ) на данный момент составляет 14,5% случаев (234/1620) (ранее 9%). Спорадический ПГПТ встречается в 83% (1592/1914) случаев. Подозрение на наследственную форму ПГПТ имеется у 326 (17%, 326/1914) пациентов: средний возраст 31,212,3 года. Данные о проведенном генетическом исследовании имеются лишь у 61 (3,2%) человека, наличие мутации в гене MEN1 подтверждено у 2,9% (55/1914), мутация в гене CDC73 у 0,3% (6/1914) (синдром HPT-JT). Рак ОЩЖ подтвержден у 1,8% (35/1914) пациентов. Хирургическое лечение проведено 64,5% (1234/1914) пациентам. Ремиссия достигнута в 94% (1160/1234) случаев. В 6% случаев зарегистрированы данные о рецидиве или персистенции заболевания после хирургического лечения. Заключение. Отмечается рост выявляемости ПГПТ в РФ по сравнению с 2016 г., что связано с активным началом работы по регистрации пациентов в регионах. На данном этапе требуется продолжение совместной доработки принципов регистрации и мониторинга, что в итоге позволит наладить систему получения информации для расчета необходимых объемов медицинской помощи данной группе.

Cervical artery dissection (CeAD) due to arterial wall weakness (dysplasia) is one of the most common causes of ischemic stroke (IS) at a young age. A rare and little known cause of CeAD is Turner's syndrome (TS)-is an inherited disease caused by completely or partially missing X chromosome. In this paper, we describe 2 female patients, aged 27 and 33 years, with genetically confirmed TS (karyotype 45X0) and internal carotid artery dissection(ICAD).TS frequency among our 304 patients with CeAD was 0.07%. Both patients had short stature, received hormone replacement therapy from the age of 14 and had arterial hypertension. In addition, the first patient suffered from hypothyroidism, osteoporosis and survived a nephrectomy for hydronephrosis. ICAD in first patient manifested by IS. MRI of the neck arteries, MRA and CTA revealed intramural hematoma, hemodynamically significant stenosis, which regressed in 4 months. In the second patient, dissection was manifested by local symptoms (Horner's syndrome, cervicocephalic pain on the dissection side). MRA and CTA revealed a precranial dissected aneurysm of the left ICA (on the side of local symptoms), fusiform expansion of the right ICA, and pathological tortuosity of both ICA. The paper discusses the cause of vasculopathy in TS. It is assumed that connective tissue damage is associated with a deficiency of biglycan - extracellular matrix protein, which interacts with collagen and elastin to strengthen the arterial wall. Biglycan gene is linked to X-chromosome which is completely or partlially missing in TS resulting in a biglycan deficiency. The role of sex hormone deficiency as a cause of arterial wall weakness is unlikely.Диссекция внутренней сонной (ВСА) и позвоночной (ПА) артерий, обусловленная слабостью (дисплазией) артериальной стенки, — одна из наиболее частых причин ишемических инсультов в молодом возрасте. Редкой и малоизвестной причиной диссекции ВСА/ПА является синдром Шерешевского-Тернера (сШ-Т) — наследственное заболевание, вызванное полным или частичным отсутствием одной из X-хромосом. В настоящей работе мы описываем 2 больных 27 и 33 лет с генетически подтвержденным сШ-Т (кариотип 45Х0) и диссекцией ВСА. Частота сШ-Т среди наших 304 больных с диссекцией ВСА/ПА составила 0,07%. Обе больные были небольшого роста, с 14—15 лет получали гормональную заместительную терапию и имели артериальную гипертонию. Кроме того, первая больная страдала гипотиреозом, остеопорозом и перенесла нефрэктомию по поводу гидронефроза. Диссекция ВСА у нее проявилась ишемическим инсультом. КТА/МРА и МРТ артерий шеи выявили прекраниальную интрамуральную гематому, гемодинамически значимый стеноз просвета артерии, который полностью регрессировал через 4 мес. У второй больной диссекция проявилась локальными симптомами (синдром Горнера, шейная и головная боль на стороне диссекции). МРА/КТА обнаружили прекраниальную аневризму левой ВСА (на стороне локальных симптомов), фузиформное расширение правой ВСА и патологическую извитость обеих ВСА. В работе обсуждается причина васкулопатии при сШ-Т. Предполагается повреждение соединительной ткани, связано с дефицитом бигликана — белка экстраклеточного матрикса, который взаимодействует с коллагеном и эластином, укрепляя артериальную стенку. Ген бигликана сцеплен с X-хромосомой, которая полностью или частично отсутствует при сШ-Т, что и вызывает дефицит бигликана. Роль дефицита половых гормонов как причины слабости артериальной стенки маловероятна.

To study clinical/laboratory signs of primary vasculitis (PV) of the internal carotid artery (ICA) and vertebral artery (VA).We examined 31 patients (23 men, 74%, mean age - 36.2±5.7 years) with ICA/VA PV verified by vessel wall contrast enhancement on black blood MRI (T1-weighted fat and blood suppressed sequences with- and without contrast injection) at the Research Center of Neurology (Moscow) from January 2012 to September 2019. Systemic vasculitis was excluded in all cases. Interleukins (IL-1β, IL-2, IL-6, IL-17), TNF-a, transforming growth factor beta 1 (TGF-β1) and basic fibroblast growth factor (bFGF) were analyzed by ELISA in 25 patients. Control group consisted of 21 healthy volunteers (12 men, 57%; mean age - 35.3±10.2 years).Clinical manifestations of ICA/VA PV included: ischemic stroke (IS) (94%), which combined with transient ischemic attacks (TIA) in 35%; isolated TIA (3%); Tolosa-Hunt syndrome (3%). Recurrent strokes were observed in 41% of patients on average in 5.3±2.1 months. Carotid artery was involved in 77%, VA - in 16%, both arteries - in 7%. Concomitant involvement of ICA/VA branches was in 19% patients. The level of arterial damage was follows: Intracranial part of arteries involved in 55%, intra-extracranial - in 35%, extracranial - in 10%. Bilateral involvement was found in 26%. Headache/neck pain in the acute IS period was observed in 21%. IS severity (NIHSS) was as follows: moderate (59%), mild (34%), moderately severe (7%). Disability after 3 months according to mRankin scale was as follows: mild (72%) moderate (21%), none (7%). The laboratory study revealed an increased levels of IL-6 (8.19±3.89 pg/ml vs 4.7±1.48 in control,ICA/VA PV has a number of clinical peculiarities. Proinflammatory cytokines produced by Th17 and Th1 CD4+ lymphocytes as well as bFGF and TGR-β1 play a role in its pathogenesis. Normal levels of IL-1β and IL-17 suggest that they are not significant in the development of isolated inflammation in ICA/PA, in contrast to systemic inflammation in giant cell arteritis, in which, according to literature data, their level increases. Isolated ICA/PA inflammation seems to be caused by transaxonal (trigeminal nerve, upper-cervical roots, autonomic nerves) spread of pathogens that initiate immune inflammation in the ICA/PA wall.Изучить клинико-лабораторные проявления первичного васкулита (ПВ) внутренней сонной (ВСА) и позвоночной (ПА) артерий.С января 2012 г. по сентябрь 2019 г. в Научном центре неврологии (Москва) был обследован 31 больной (средний возраст 36,2±5,7года, 23% мужчины) с ПВ ВСА/ПА, верифицированным по накоплению их стенкой контрастного вещества при МРТ в режиме T1 dark-blood+fat-sat. Системный васкулит был исключен во всех случаях. Исследование интерлейкинов (ИЛ-1β, ИЛ-2, ИЛ-6, ИЛ-17), фактора некроза опухолей альфа (ФНО-α), трансформирующего фактора роста β1 (ТФР-β1) и основного фактора роста фибробластов (оФРФ) с помощью ELISA было проведено у 25 больных. Контрольную группу составил 21 здоровый (12 (57%) мужчин; средний возраст 35,3±10,2 года).Клинические проявления ПВ включали ишемический инсульт (94%), который в 35% сочетался с преходящими нарушениями мозгового кровообращения (ПНМК); изолированное ПНМК (3%); синдром Толоса—Ханта (3%). Рецидивы инсульта в среднем через 5,3±2,1 мес отмечены у 41% больных. Поражение ВСА отмечено в 77% случаев, ПА — в 16%, сочетанное — в 7%. Сопутствующее вовлечение ветвей ВСА/ПА имелось у 19% больных. Интракраниальный отдел артерий поражался в 55% случаев, интра-экстракраниальный — в 35%, экстракраниальный — в 10%. Двустороннее поражение отмечено в 26% случаев. Головная/шейная боль в остром периоде инсульта наблюдалась в 21% случаев. Тяжесть инсульта (по шкале NIHSS) была умеренной (59%), легкой (34%), умеренно тяжелой (7%). Степень нетрудоспособности через 3 мес по модифицированной шкале Рэнкин соответствовала легкой (72%), умеренной (21%) или отсутствовала (7%). Лабораторное исследование выявило повышение ИЛ-6 (8,19±3,89 пг/мл против 4,7±1,48 пг/мл в контроле,ПВ ВСА/ПА имеет ряд клинических особенностей. В его патогенезе имеет значение выработка провоспалительных цитокинов CD4+ лимфоцитами, относящимися к подвиду Th17 и Th1, а также продукция оФРФ и ТФР-β1. Нормальный уровень ИЛ-1β и ИЛ-17 позволяет предполагать, что они существенно не значимы в развитии изолированного воспаления ВСА/ПА в отличие от системного воспаления при гигантоклеточном артериите, при котором, по данным литературы, их уровень повышен. Изолированное поражение ВСА/ПА, по-видимому, обусловлено трансаксональным (тригеминальный нерв, верхнешейные корешки, вегетативные нервные волокна) распространением патогенов, инициирующих иммунное воспаление в стенке ВСА/ПА.

Pseudohypoparathyroidism (PGPT) - Albright's hereditary osteodystrophy is a sex-related disease rarely seen in medical practice. Inherited by the dominant type. It is caused by insensitivity of the kidneys and bones to parathyroid hormone (PG), which is secreted in normal or slightly elevated amounts [6, 10]. GH may not have a specific effect on cells due to pathology of GH receptors, impaired expression of information RNA synthesis by an unchanged hormone-receptor complex, the presence of pathological components of adenylate cyclase, pathological AMP-dependent protein kinase, or a defect in the synthesis of kinase substrates in the presence of circulating GH antagonists [5, 10 ].

Aim of this study was to estimate the markers of mineral turnover and BMD in young women with the central hypogonadism, to compare them with healthy young women and healthy postmenopausal women of middle/advanced age. Materials and methods. One hundred seventy women were included in the study: 73 patients with the central hypogonadism (isolated hypogonadism n=35, hypopituitarism n=38), age of 25 [21.2; 30.5] y.o., amenorrhea duration 5 [2.3; 10.1] years; 47 healthy women with regular menstrual cycles, age 24 [23.1; 28.0] y.o., and 50 healthy women with natural menopause, age 56 [53; 58] y.o., menopause duration 6.0 [2.1; 10.0] years. Groups did not differ by age. Results. In patients with central female hypogonadism concentrations of calcium and alkaline phosphatase were significantly higher than in healthy women of similar age, however did not differ from the parameters in postmenopausal women. T-scores

Despite high prevalence and severity of complications, osteoporosis (OP) in Russia is still not recognized as a socially significant disease and there are no common standards for prevention of OP and associated fractures. The aim of the study was to investigate the adherence of healthy females to prevention measures of OP and the affecting it factors to develop the principles governing the preventive strategy of OP. Methods. The study is performed in Moscow Region (MR) in a form of a questionnaire survey in the population of adult healthy women aged >20 years. The data are obtained using "Questionnaires on osteoporosis prevention" and "The test of knowledge in the field of osteoporosis" developed in Clinical Research Institution of Moscow Region named after M.F. Vladimirsky. Study comprised 1712 female aged from 20 till 87 years (median 55,0 years [45,0; 44,0]) living in 16 districts and 11 cities of MR. Taking into account the quality of the filling of questionnaires, data provided by 1631 women were included in the statistical analysis. Results. It is established that 31% of female inhabitants of MR are engaged in OP prevention, using for this purpose mainly calcium-containing drugs (64,3%) and increased consumption of calcium in food (59,8%). 93% of these women get preventive therapy at their own expense, spending on them on the average 200 rub a month, and preventive medication has appeared more saving than non-pharmacological preventive measures. Socially active working women at the age of 50-69 years are motivated on prevention of OP better than others. Level of the OP awareness and undertaking densitometry screening also directly influence adherence to OP prevention.

Objectives. To analyze clinical data of the first domestic zoledronic acid generic preparation for the treatment of osteoporosis (Rezoclastin PS., 5 mg). Materials and Methods. The study included data from 123 patients (118 women and 5 men) treated with Rezoclastin PS 5 mg in medical centers of Moscow, St.-Petersburg and Krasnodar for primary (91.8%) or secondary(8.2%) osteoporosis, with mean age 27-78 years (61,4 ± 9,1 years). 56.6% of patients had severe osteoporosis with compressive deformations of vertebrae. Duration of medical therapy ranged from 6 to 24 months. Results. Study of changers in bone resorption marker CTx (n = 10) showed decline by 78% after 3 months of treatment compared to baseline (p = 0.027). BMD at the lumbar segment of the spine L 1-L 4 increased after 12 months by 3.4% (p

Women of reproductive age with acromegaly can be interested in pregnancy, even when the pituitary tumor keeps rather big sizes, or control over secretion of GH and IGF-1 has not been reached. In this regard doctors often meet questions of acromegaly influence on the course of pregnancy. Now a little more than 100 cases of pregnancy at acromegalic female patients were described. In article the short review of the cases published in literature is carried out, and a new own clinical case of pregnancy at the patient with acromegaly is represented. The young woman (24 y.o.) received treatment with octreotide prolonged release in a dose 20/40 mg after non-radical adenomectomy for somatoprolactinoma, on this background she became pregnant, and continued medical treatment during all pregnancy. There were no complications during pregnancy and/or pathology of fetal formation. In women with acromegaly treatment with somatostatin analogs is not the indication for pregnancy interruption. It is possible to stop this treatment for the period of pregnancy as such break in treatment is safe for mother and fetus in most cases according to the literature. However if the estimated risk of further growth of a pituitary tumor is high, treatment can be continued during pregnancy.

Aim of the study was to investigate the features of glucose metabolism in patients with acromegaly depending on treatment. Materials and methods: 63 patients with acromegaly: 29 patients with newly diagnosed acromegaly, 25 patients receiving somatostatin analogs (SSA) therapy and 9 patients underwent surgical treatment. Patients with earlier revealed diabetes mellitus (DM) were not included. The characterization of glucose metabolism was carried out by means of an assessment of fasting insulin plasma levels (FIP) and indexes of an insulinresistance (IR) HOMA-R and an insulinsensivity (IS) MATSUDA. Results: In patients receiving SSA therapy the carbohydrate metabolism disorders (CMD) were revealed in 92%, among newly diagnosed acromegalic patients – in 62% and in patients after surgical treatment in 33% of cases. In newly diagnosed acromegalic patients index MATSUDA was 3,5 times lower than in patients receiving SSA therapy, and 4 times lower than in patients after surgical treatment ((р=0,001). НОМА-R in newly diagnosed acromegalic patients was 3,7 times higher than in other groups, p=0,003 and the level of FIP was 4,3 times higher than in patients receiving SSA therapy and 2,9 times higher than in patients after surgical treatment (р=0,001). The index MATSUDA in newly diagnosed acromegalic patients with early CMD was 2,9 times lower, р=0,006, НОМА-R – 2,5 times higher, р=0,025 and FIP 2,4 times higher, р=0,039 than in patients with early CMDs without acromegaly. Conclusions: In patients with newly diagnosed acromegaly in the absence of CMDs increase in IR is compensated by hyperinsulinemia. In patients, receiving SSA therapy the IR decreases, however suppression of secretion of insulin leads to increase in percentage of DM in this group. After surgical treatment IR decreases against higher level of fasting plasma insulin that leads to normalization of carbohydrate metabolism. Keywords: Acromegaly, secondary diabetes mellitus, insulinresistance, MATSUDA, HOMA-R.