Search

[English] Due to continuous aging of population and increase in the number of elderly people, osteoporosis became socially significant disease leading to disability, increasing mortality and thereby putting an additional burden on the public healthcare system. Screening to identify groups with a high probability of fracture is recommended using the FRAX® Tool for all postmenopausal women and men over 50 years old (А1). In the presense of major pathological fractures (hip, spine, multiple fractures) it is recommended to diagnose osteoporosis and prescribe treatment regardless of the results of spine and hip double X-ray absorptiometry (DXA) or FRAX® (B2). It is recommended to evaluate C-terminal telopeptide when prescribing antiresorptive therapy and procollagen type 1 N-terminal propeptide (P1NP) when prescribing anabolic therapy to patients receiving osteoporosis treatment at baseline and 3 months after the start of therapy in order to assess the effectiveness of treatment early and adherence to the therapy (А2). It is recommended to diagnose osteoporosis and prescribe treatment to patients with high individual 10-year probability of major pathological fractures (FRAX®) regardless of the results of spine and hip DXA (В3). It is recommended to diagnose osteoporosis and prescribe treatment with a decrease in BMD, measured by DXA, by 2.5 or more T-score standard deviations in femoral neck, and/or in total hip, and/or in lumbar vertebrae, in postmenopausal women and men over 50 years old (А2). It is recommended to prescribe bisphosphonates, denosumab or teriparatide to prevent pathological fractures and increase BMD in patients with postmenopausal osteoporosis, osteoporosis in men, glucocorticoid-induced osteoporosis (А2). When the clinical effect of therapy in osteoporotic patients without pathological fractures is achieved (BMD T-score > -2.0 SD in femoral neck and absence of new fractures), it is recommended to interrupt bisphosphonates therapy for 1-2 years with subsequent follow-up (B2). In patients with vertebral fractures, hip fractures or multiple fractures, it is recommended to continue ceaseless long-term treatment of osteoporosis (В3). All drugs for the treatment of osteoporosis are recommended to be prescribed in combination with calcium and cholecalciferol (А2). In order to reduce the risk of recurrent fractures by prescribing osteoporosis therapy timely and maintaining long-term follow-up of patients over 50 years old with pathological fractures, it is recommended to create Fracture Liaison Services (В2). [Русский] В связи с непрерывным старением населения и увеличением количества лиц старшего и пожилого возраста остеопороз стал социально значимым заболеванием, приводя к инвалидности, повышая уровень смертности и создавая тем самым дополнительную нагрузку на систему здравоохранения. Скрининг для выявления групп с высокой вероятностью переломов рекомендован с использованием Алгоритма FRAX® среди всех женщин в постменопаузе и мужчин старше 50 лет (А1). При наличии патологических переломов крупных костей скелета (бедренной кости, тел(а) позвонков(-а), множественных переломов) в анамнезе рекомендовано устанавливать диагноз остеопороза и назначать лечение независимо от результатов рентгеноденситометрии поясничного отдела позвоночника и проксимального отдела бедренной кости или FRAX® (B2). Рекомендуется определение С-концевого телопептида при назначении антирезорбтивной терапии и исследование уровня N-терминального пропептида проколлагена 1-го типа (P1NP) при назначении анаболической терапии пациентам, получающим лечение остеопороза, исходно и через 3 месяца от начала терапии с целью ранней оценки эффективности лечения и приверженности к терапии (А2). Рекомендовано устанавливать диагноз остеопороза и назначать лечение пациентам c высокой индивидуальной 10-летней вероятностью основных патологических переломов (FRAX®) независимо от показателя рентгеноденситометрии поясничного отдела позвоночника и проксимального отдела бедренной кости (В3). Рекомендовано устанавливать диагноз «остеопороз» и назначать терапию при снижении МПК, измеренной при DXA, на 2,5 и более стандартных отклонения по Т-критерию в шейке бедренной кости, и/или в целом в проксимальном отделе бедренной кости, и/или в поясничных позвонках у женщин в постменопаузе и у мужчин старше 50 лет (А2). Для предупреждения патологических переломов и повышения МПК у пациентов с постменопаузальным остеопорозом, остеопорозом у мужчин, глюкокортикоидным остеопорозом рекомендуется прием бисфосфонатов, деносумаба или терипаратида (А2). Пациентам с остеопорозом без патологических переломов при достижении клинического эффекта терапии (МПК до -2,0 SD по Т-критерию в шейке бедренной кости и отсутствие новых переломов) рекомендовано на 1-2 года прекратить терапию бисфосфонатами с последующим динамическим наблюдением (B2). Пациентам с переломами тел позвонков, бедренной кости или множественными переломами в анамнезе рекомендуется продолжать непрерывное длительное лечение остеопороза (В3). Все препараты для лечения остеопороза рекомендуется назначать в сочетании с препаратами кальция и колекальциферола (А2). С целью своевременного назначения терапии остеопороза и обеспечения длительного наблюдения за пациентами в возрасте 50 лет и старше с патологическими переломами для снижения риска повторных переломов рекомендуется создавать Службы профилактики повторных переломов (В2).

Primary hyperparathyroidism (PHPT) is an endocrine disorder of parathyroid glands characterized by excessive secretion of parathyroid hormone (PTH) with an upper normal or elevated blood calcium level. Classical PHPT refers to a symptomatic, multi-system disorder, wich can lead to a significant decrease in the quality of life, disability of patients, and even an increased risk of premature death. Hypercalcemia and the catabolic effect of PTH on various cells are considered as the main pathogenetic mechanisms of the PHPT associated complications. In the last two decades, there has been an increase in the incidence of PHPT, mainly due to the mild forms of the disease, primarily due to the routine calcium screening in North America, Western Europe and, Asia. High prevalence of the disease, as well as the variety of clinical manifestations, cause the attention of different specialists - physicians, rheumatologists, urologists, nephrologists, cardiologists and other doctors. This review cover the main issues of Russian guidelines for the management of PHPT, approved in 2020, including laboratory and instrumental methods, differential diagnosis, surgical and conservative approach, short-term and long-term follow-up. This guidelines also include the recommendations for special groups of patients with hereditary forms of PHPT, parathyroid carcinoma, PHPT during pregnancy.

Despite the implementation of screening programs for early diagnosis of congenital disorders, both in the Russian Federation, as well as worldwide, there are still cases of late diagnosis of congenital adrenal cortical hyperplasia (CAH), where after long-standing virilization clinicians often face the need of gender assignment, considering not only the medical indications, but also the patient's self-identification. In this article, we present a rare clinical case of patient with hypertonic type of CAH diagnosed in adulthood. At birth, the patient had ambiguous genitalia; the diagnosis of CAH was made, and the patient was assigned to female gender. However, due to some social factors, the patient was not followed up and received no treatment. The lack of treatment resulted in progression of the virilization, disturbance of the patient's gender self-identification (the female patient considered himself male), final short stature and arterial hypertension. The patient attended endocrinology clinic for the first time at the age of 19. The diagnosis of hypertensive type of CAH was based on clinical manifestation of the virile syndrome combined with hyporeninemic arterial hypertension, as well as the results of multisteroid blood analysis with identification of steroid cortisol precursors and the results of the genetic testing for CYP11B1 gene mutation. Administration of glucocorticoid replacement therapy after the late diagnosis of hypertonic type of CAH did not resolve all of the patient's concerns. The disorder of the gender self-identification in a female patient resulted in the change of the passport gender to the male one, with surgical correction in accordance to the chosen gender and replacement androgen therapy. Persistent arterial hypertension required additional anti-hypertensive treatment. This clinical case highlights that psychosocial, ethnic factors and religious factors should be taken into consideration, as well as the multidisciplinary involvement of endocrinologists, psychiatrists, andrologists and gynecologists is necessary for the management of such patients.

Parathyroid carcinoma (PC) is a rare neoplasm with a prevalence of only 0.005 % of all malignancies, and less than 1 % among patients with primary hyperparathyroidism. The diagnosis can be challenging due to the rarity of the disease, similarity of clinical features to parathyroid adenoma, and lack of reliable morphological signs of the tumor. For metastatic cancer of the parathyroid gland does not exist standard drug therapy due to the rare occurrence of this disease. Chemotherapy and radiotherapy failed to show any significant effect on the course of the disease. We report the case of a 27-year old woman with PC previously misdiagnosed as benign thyroid nodule and multiple lung metastases. After the immunohistochemical study and confirm of diagnosis PC the patient was treatment with multikinase inhibitor sorafenib successfully.

The review of literature details the issues of genetics, the specific features of inheritance, the clinical picture and treatment of pseudohypoparathyroidisms (PHPT). In practice, clinicians more frequently deal with type 1 PHPT and the diagnosis of this type creates no significant problems. However, despite the low prevalence of the other types of PHPT - 1b, 1c, and 2, the diseases may run with noticeably clinical symptoms and present a significant problem in the context of diagnosis and treatment. This review may be of concern to both clinicians and geneticists who are interested in this problem.В обзоре литературы подробно освещены вопросы генетики, особенностей наследования, клинической картины и лечения заболеваний группы псевдогипопаратиреоза (ПГПТ). В практической деятельности клиницисты чаще имеют дело с типом 1а ПГПТ, и диагностика этого типа не вызывает значительных сложностей. Однако, несмотря на малую распространенность остальных типов ПГПТ - 1b, 1с и 2, заболевания могут протекать с выраженной клинической симптоматикой и представлять значительную проблему в плане диагностики и лечения. Данный обзор может представлять интерес как для клиницистов, так и для генетиков, интересующихся этой проблемой.

Background: Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome is a type hypercorticism caused by ectopic production of ACTH and/or its precursors by neuroendocrine tumors (NET) of various localization, such as lung and thymus carcinoids, less frequently those of pancreas and gastrointestinal tract, medullary thyroid cancer, pheochromocytoma, small cell lung cancer, as well as some other tumor types. The wide spectrum of tumors associated with the ectopic ACTH syndrome (EAS) makes their diagnosis and treatment a complicated issue. Materials and methods : The study was done with surgical and diagnostic biopsy samples from 60 patients who had tumors with EAS, 36 (60%) of them being bronchopulmonary carcinoids, 10 (16.7%) thymus carcinoids, 5 (8.3%) pancreatic NETs, 3 (5%) medullary thyroid cancers, 3 (5%) NETs of unknown primary localization, and NET of appendix, cecum and pheochromocytoma (one case of each, i.e. 1.7%, 1.7%, 1.7%). There were 38 female and 22 male patients (1.72:1), with their mean age of 39 ± 14 years (range, 16 to 77 years). We analyzed their clinical data, as well as the results of morphological and immunohistochemical examination of the tumors. Results : According to the World Health Organization classification (2015), typical carcinoids (TC) of the lung were found in 77.8% (28/36) of the bronchopulmonary tumors. 22.2% (8/36) of the bronchopulmonary tumors and all thymus tumors (100%, n = 10) were classified as atypical carcinoids (ATC). Four pancreatic NETs were classified as Grade 2 (G2), 1 as G3, and NETs of the cecum and appendix as G1 and G2, respectively. At present, 29 (48%) patients are living with no relapse (mean age 39 ± 13.5 years), whereas 22 (37%) of patients developed a relapse (mean age 35 ± 13 years), and 15 (25%) of them died, regardless of the age of the patients The history of the disease was not traced in 4 cases. 5-year relapse-free survival of patients with TC of the lung was 85.7% (24/28), of those with ATC 25% (2/8), with ATC of the thymus and medullary thyroid cancer 0%. Mortality from to TC of the lung for the entire follow-up period was 3.6% (1/28), to ATC 12.5% (1/8), to ATC of the thymus and for medullary thyroid cancer 62.5% and 100%, respectively. The patients with NET of the cecum, appendix and pheochromocytoma are alive without progression for 4, 5 and 6 years, respectively. Therefore, the lowest 5-year survival of patients with EAS was observed in medullary thyroid cancer, pancreatic NET and thymus carcinoids: in 100% (3/3), 75% (3/4), and 57.1% (4/7), respectively. Conclusion: The most unfavorable prognostic factors in the EAS are the localization of tumors in the thymus, pancreas and thyroid gland. This indicates the necessity of a differentiated treatment approaches to patients with this syndrome.

A review of the literature is devoted to the issue of secondary hyperparathyroidism (SHPT) with underlying chronic renal failure. Initial manifestations of the disease can be observed even with a minimal decrease in the rate of glomerular filtration and are pathophysiologically associated with violations of normal metabolism of vitamin D with its conversion into D-hormone. SHPT pathogenesis is based on the D-hormone deprivation and triggering of pathophysiology mechanisms of bone remodelling, increased FRP-23, PTH, changes in the serum levels of calcium and phosphorus, which may further lead to significant changes in the bone tissue structure and cardiovascular complications. Active metabolites of vitamin D and vitamin D analogues (Paricalcitol) were developed for the replacement of active forms of vitamin D in conditions of D-hormone deficiency. Due to its chemical structure and pharmacokinetics, Paricalcitol is able to block the synthesis of parathyroid hormone to a greater extent and increase the absorption of calcium and phosphorus in the intestine to a lesser extent, which gives it advantages over pre-existing active metabolites

Представлено краткое изложение клинических рекомендаций по диагностике и лечению остеопороза, разработанных членами Российской ассоциации эндокринологов при участии членов Российской ассоциации по остеопорозу (ревматологов, травматологов, терапевтов, гинекологов-эндокринологов). Рекомендации разработаны с позиций доказательной медицины, в соответствии с требованиями к составлению клинических рекомендаций Минздрава России, опубликованными в 2016 г. Значительное место в изложении отведено диагностике остеопороза у взрослых, дифференциальной диагностике с другими метаболическими заболеваниями скелета, а также принципам патогенетического лечения остеопороза. Клинические рекомендации полезны будут как врачам общей практики, так и специалистам, прежде всего эндокринологам, ревматологам, травматологам-ортопедам, гинекологам, нефрологам, гастроэнднрологам и неврологам, так как остеопороз является мультифакториальным и мультидисциплинарным заболеванием

The article describes two clinical cases of severe primary hyperparathyroidism (PHPT) caused by parathyroid carcinoma in young female patients who underwent molecular genetic testing to rule out the hereditary forms of PHPT. In both patients, heterozygous germline nonsense mutations of tumor suppressor gene CDC73 encoding parafibromin (p.R91X and p.Q166X) were identified using next-generation sequencing with Ion Torrent Personal Genome Machine (Thermo Fisher Scientific - Life Technologies, USA). It is the first description of CDC73 mutations in Russia, one of the mutations is described for the first time in the world. Identification of germline mutations in the CDC73 gene in patients with PHPT necessitates regular lifelong screening for other manifestations of hyperparathyroidism-jaw tumor syndrome (HPT-JT), PHPT recurrence due to parathyroid carcinoma as well, and identification of mutation carriers among first-degree relatives.Представлено описание двух клинических случаев первичного гиперпаратиреоза (ПГПТ) тяжелого течения у молодых больных раком околощитовидной железы, которым проведено молекулярно-генетическое исследование для исключения наследственных форм ПГПТ. Методом высокопроизводительного параллельного секвенирования на полупроводниковом секвенаторе Ion Torrent Personal Genome Machine (Thermo Fisher Scientific - Life Technologies, США) у обеих пациенток выявлены герминальные гетерозиготные нонсенс-мутации в гене - супрессоре опухолевого роста CDC73, кодирующем белок парафибромин: p.R91X и p.Q166X. В России мутации в указанном гене описываются впервые, одна из мутаций описывается впервые в мире. Выявление герминальных мутаций в гене CDC73 у пациентов с ПГПТ требует пожизненного регулярного профилактического обследования для ранней диагностики компонентов синдрома гиперпаратиреоза с опухолью челюсти (hyperparathyroidism-jaw tumor syndrome - HPT-JT), рецидива ПГПТ после хирургического вмешательства, в том числе вследствие рака околощитовидной железы, а также позволяет выявить носителей мутаций среди родственников первой линии родства.Представлено описание двух клинических случаев первичного гиперпаратиреоза (ПГПТ) тяжелого течения у молодых больных раком околощитовидной железы, которым проведено молекулярно-генетическое исследование для исключения наследственных форм ПГПТ. Методом высокопроизводительного параллельного секвенирования на полупроводниковом секвенаторе Ion Torrent Personal Genome Machine (Thermo Fisher Scientific — Life Technologies, США) у обеих пациенток выявлены герминальные гетерозиготные нонсенс-мутации в гене — супрессоре опухолевого роста CDC73, кодирующем белок парафибромин: p.R91X и p.Q166X. В России мутации в указанном гене описываются впервые, одна из мутаций описывается впервые в мире. Выявление герминальных мутаций в гене CDC73 у пациентов с ПГПТ требует пожизненного регулярного профилактического обследования для ранней диагностики компонентов синдрома гиперпаратиреоза с опухолью челюсти (hyperparathyroidism-jaw tumor syndrome — HPT-JT), рецидива ПГПТ после хирургического вмешательства, в том числе вследствие рака околощитовидной железы, а также позволяет выявить носителей мутаций среди родственников первой линии родства.

Microribonucleic acids (miRNAs) are a class of noncoding RNAs that regulate posttranscriptional gene expression. These molecules are regulators of cell proliferation, metabolism, apoptosis, and differentiation. MiRNAs are not degraded by RNAases and their concentrations can be measured in different body fluids, including serum. The expression of miRNAs varies in intact tissues and tumors, including pituitary adenomas. Pituitary tumors are encountered in 22.5% of the population and, in a number of cases, may be asymptomatic, but in case of invasion or/and hormone overproduction, their clinical presentation is severe with multiple symptoms leading to disability and even death. The mechanisms for the development and progression of pituitary tumors and the markers for remission and recurrence have not been adequately investigated. This literature review discusses the biological significance of miRNAs in pituitary tumors and the potential value of circulating miRNAs as biomarkers.Микрорибонуклеиновые кислоты (микроРНК) - класс некодирующих РНК, которые регулируют посттранскрипционную экспрессию генов. Эти молекулы являются регуляторами, контролирующими пролиферацию, метаболизм, апоптоз и дифференцировку. МикроРНК не подвержены разрушению РНКазами и их концентрация может быть измерена в различных биологических жидкостях, в том числе сыворотке крови. Экспрессия микроРНК в опухолях и здоровых тканях, в том числе в аденомах гипофиза, различна. Опухоли гипофиза встречаются у 22,5% населения и в ряде случаев могут протекать бессимптомно, но при их инвазивном росте и/или гормональной активности новообразования развиваются тяжелые многосимптомные заболевания, приводящие к инвалидности и смерти. Механизмы возникновения и прогрессирования опухолей гипофиза, маркеры ремиссии и рецидива не достаточно изучены. В настоящем обзоре литературы обсуждается биологическое значение микроРНК в опухолях гипофиза и потенциальное значение циркулирующих микроРНК как биологических маркеров.

This review discusses the recent evidence showing that the skeleton itself produces at least two hormones: fibroblast growth factor 23 (FGF23) and osteocalcin. FGF23 is secreted by osteocytes in bone and acts on the kidney to inhibit 1-alpha-hydroxilation ofvitamin D and promote phosphorous excretion. The affinity of FGF23 to FGF receptor is low, but FGF23 binds to FGF receptor-Klotho complex with more affinity. Therefore, Klotho determines the kidney-specific action of FGF23. Increase in FGF23 or Klotho levels due to genetic defects or ectopic production results in low serum phosphorous levels in humans. Contrary to this, low FGF23 or Klotho levels lead to hypophosphatemia and ectopic calcification. Mouse genetics studies revealed that osteoblast product, osteocalcin, in its undercarboxylated stage acts on the pancreatic beta-cells to enhance insulin production and on peripheral tissues to increase glucose utilization as a result of increased insulin sensitivity and to reduce visceral fat. In addition to this, undercarboxylated osteocalcin may also have another hormonal role, this time as a mediator of testosterone secretion. Osteocalcin was shown to induce testosterone production in Leydig cells of the testes both in ex vivo and in vivo studies. In both localizations, at the pancreas and at the testes osteocalcin acts through the GPCR6A receptor, this activates the cAMP response element-binding protein signaling pathway. Thus, this review reports the recent studies indicating bone ’s role as an endocrine organ.

Clinical symptoms of vitamin D deficiency may be quite misleading and masked as rare hereditary syndromes. We describe a family, sister and brother presented with the pain in lower extremities at the age of 14 and 16years accordingly, with severe vitamin D deficiency that was misdiagnosed in the course of the disease with pseudohypoparathyroidism and 1-alpha-hydroxylase deficiency. They benefited from treatment with alfacalcidol and ossein-hydroxyapatite complex supplement which were further discontinued due to socioeconomic factors. At presentation after 2 years without treatment extremely low 25(OH)D levels were revealed. The patients' family history was remarkable for the same clinical features in mother and her Indian ancestry. Thus we describe an uncommon manifestation of severe vitamin D deficiency in a familial setting which emphasizes the necessity of vitamin D testing in calcium or parathyroid disorders.

Aim. To evaluate the effect of parathyroidectomy and cinacalcet on anemia, lipid profile and blood pressure (BP) in uremic hyperparathyroidism.Material and methods. Uremic patients (n=39) treated with hemodialysis and having secondary hyperparathyroidism were included into the study. Radical parathyroidectomy was performed in 21 patients, 18 patients were treated with cinacalcet. BP measurement, determination of blood levels of albumin, total calcium, phosphorus, total cholesterol (TC), low (LDL) and high density lipoproteins, triglycerides, intact parathyroid hormone, and hemoglobin were performed in all patients initially and during treatment. Doses of antihypertensive and erythropoiesis-stimulating agents were also assessed.Results. Calcium-phosphorus metabolism indices improved after 6 months of cinacalcet therapy and parathyroidectomy (pConclusion. The pleiotropic effects (reduction in BP and atherogenic lipids levels, as well as decrease in anemia resistant to the action of erythropoiesis-stimulating agents) were found after parathyroidectomy and cinacalcet therapy additionally to calcium-phosphorus metabolism improvement.

The paper presents data on the role of vitamin D and calcium in the function of many human organs and tissues. Lifestyle, dietary preferences, and insufficient physical activity contribute to the high prevalence of vitamin D and calcium deficiencies in the adult population of Russia, causing different diseases and abnormalities. The authors have worked out recommendations for the preventive use of vitamin D and calcium in healthy population, give consumption rates for these substances, and describe the clinical and laboratory signs of vitamin D deficiency and indications for screening. They also propose treatment regimens for vitamin D deficiency and depict the signs of intoxication in overdose. Particular emphasis is laid on the place of vitamin D and calcium in the therapy of osteoporosis.

Objective - to evaluate the value of trabecular bone score and risk factors of fractures in patients with Cushing’s syndrome (CS). Material and methods. One hundred eighty two patients with laboratory-confirmed Cushing’s syndrome were enrolled. All patients underwent measurement of bone mineral density (BMD) at the lumbar spine (LI-LIV), femoral neck and total hip using DXA Prodigy (GEHC Lunar, Madison, WI, USA). Trabecular bone score (TBS) was assessed retrospectively on the basis of already existing DXA images using software TBS iNsight software v2.1 (Medimaps, Merignac, France). Each patient was interviewed for the presence of low-traumatic fractures during the active stage of the disease. A lateral X-ray of the thoracic and lumbar spine ThIV-LV was performed to estimate vertebral fractures. Twenty-four hours urinary free cortisol (24hUFC) was measured by imunochemiluminescence assay VITROS ECi with the preliminary extraction with diethyl ether (reference values 60-413 nmol/24 h). Results. Among 182 patients with CS (149 women, 33 men), Cushing’s disease was confirmed in 151 cases, 9 patients diagnosed with benign adrenal tumor and 22 - ACTH-ectopic syndrome. The median of age - 35 (Q25-Q75 27-49) years, body mass index - 29 (26-33) kg/m2, 24hUFC - 1760 (985-2971) nmol/24h. Fractures were confirmed in 80 (44%) cases, 70 patients suffered from vertebral fractures, which were multiple in 53 cases; 23 patients had non-vertebral fractures. Median of trabecular bone score was 1.205 (1.102-1.307), which is much lower than expected in healthy volunteers (>1.350), while the decrease in bone mineral density (BMD) did not correspond to the severity and prevalence of osteoporosis fractures: LI-LIV Z-score - 1.7 (2.5-0.73); femoral neck Z-score - 1 (-1.6- -0.4). However, when using binary logistic regression analysis (adjusted for sex, age, body mass index, bone mineral density, trabecular bone score and 24hUFC) revealed that the most significant predictor of fracture is high levels of 24hUFC (p=0.001) . The high prevalence of vertebral fractures in patients with CS most likely influenced the results of trabecular bone score and its ability to predict fractures. Conclusion. Patients with active CS have low trabecular bone score, rather than bone mineral density, which reflects deterioration in bone microarchitecture. The low-traumatic fracture occurrence depends on the severity of CS reflected in 24hUFC levels.

The article presents a literature review summarizing the contemporary data on the effects of drug therapy on various parameters of renal osteodystrophy: phosphate binders, vitamin D preparations, bisphosphonates, denosumab, and calcimimetics. We discuss the results of pilot study of the efficacy of teriparatide and denosumab on parameters of bone metabolism in patients with chronic kidney disease.

This study estimates the recovery of bone mineral density (BMD), markers of bone remodeling and quality of life in patients with endogenous Cushing’s syndrome (CS) after 12 months of achieving remission. Materials and methods: 21 patients with CS were prospectively evaluated at active stage of the disease and after being in a full remission (substitutional therapy with hydrocortisone or normal 24 hours urinary free cortisol (24h UFC) and late-night cortisol) during 12 months.A thoracic and lumbar X-ray was performed to reveal vertebral fractures. Bone mineral density (BMD) was measured by DXA ((Prodigy, Lunar, GE, USA). The level of 24h UFC was measured on a VitrosECi. Late-night serum cortisol and markers of bone remodeling were assayed by ECLIA Cobas e601 Roche. Patients fulfilled EQ-5D, ECOS-16 questionnaires and performed "up-and-go ”, "tandem ” and "chair-rising ” tests. Results: Among enrolled patients 17 (80%) were females and 4 (20%) - males; median of age (Q25-Q75) - 41 (33-49) years old; in 10 cases (48%) low traumatic fractures were diagnosed: 7 patients suffered from vertebral fractures; in 3 cases - ribs fractures. After the achieving remission no new fractures were registered and significant improvement in Z-score was reviled at all regions: L1-L4 -1,8 [-2,6; -0.5] at active stage vs -1,2 [-2,2; -0.5] after 12 months of remission (p=0.05); Neck Z-score -0,9 [-1,7; - 0,8] vs -0,7 [-1,6; -0,3], (p=0,003). The level of both bone metabolism markers increased: osteocalcin from 8,2 (6,912,0) to 22,7 (12,1-36,5) ng/ml (p=0,01) and CTx from 0,35 (0,22-0,63) to 0,7(0,28-1,05) ng/ml (p=0,01); whereas 24hUFC decreased from 1449 (926,4-2371) nmol/24h to 66,4(54,2-76,4) nmol/24h (p=0,01). The quality of life significantly improved at all dimensions if measured by ECOS-16. According to the EQ-5D patients suffered less from pain 1,35 (0,49) vs 1,12 (0,34), (p=0,04) and reported the improvement in their health (visual analogue scale) from 49 (18,9) to 68 (10,9), (p=0,004), but did not differ in others dimensions. Although 100% of patients admitted the improvement in their functional ability, the difference in functional tests did not reach statistical significance. Conclusions: Achieving the remission of CS improves BMD and quality of life in patients with CS. However, longer time is needed for full recovery, including the functional performance.

Постепенное увеличение продолжительности жизни человека, стремление к улучшению ее качества требует улучшения результатов профилактики и лечения остеопороза. Основную часть лиц, подверженных ОП, составляют женщины в менопаузе. Разносторонний подход к проблеме восстановления баланса костного метаболизма привел к внедрению в практику препаратов с различными механизмами действия. В последнее время крайне важным аспектом является повышение приверженности к лечению пациентов, получающих многолетнее лечение остеопороза. Инновационным шагом является внедрение в практику применения нового препарата моноклонального человеческого антитела к лиганду RANK деносумаб. В обзоре представлены результаты сравнительных многоцентровых слепых рандомизированных исследований эффективности деносумаба и пероральных бисфосфонатов. Показано более выраженное достоверно влияние деносумаба (подкожно 60 мг 1 раз в 6 мес.) на прирост МПК во всех отделах скелета (как богатых трабекулярной, так и кортикальной костной тканью), значимое снижение риска переломов при хорошей переносимости лечения.

In this critical review, we have summarized the specific evidence on ibandronic acid (Bonviva) efficacy, tolerability, feasibility and safety acquired from randomized controlled clinical trials, meta-analyses, bridging trials, long-term extension studies, observational studies and clinical experience. We have paid special attention to reviewing the surrogate endpoints, which are routinely used in clinical practice to estimate the treatment efficacy as well as those high technology methods that are currently used in research centers to predict anti-fracture efficacy. Although the only registered indication for Bonviva in Russia is for postmenopausal osteoporosis, we have reviewed the available clinical trials on the efficacy of ibandronic acid to prevent postmenopausal osteoporosis, to treat low bone mass in males, to treat and prevent glucocorticoid induced osteoporosis and other types of secondary osteoporosis and, in particular, in patients after organ transplantation. In addition to this, we have extensively discussed the currently available data on the safety of pharmacologic treatment for osteoporosis.

The objective of this study was to investigate the prevalence of low traumatic fractures, the factors influencing fractures in endogenous Cushing’s syndrome (CS) of various etiologies and their contributions into functional abilities and quality of life in patients with CS. Materials and methods: the retrospective data of patients, who had received treatment due to endogenous CS, (2001-2011), was evaluated. All enrolled patients underwent standard spinal radiographs in lateral positions of the vertebrae Th4-L4. Recent low traumatic non-vertebral fractures were recorded in the medical cards. Bone mineral density (BMD) was measured by DXA GE Lunar Prodigy. Serum samples on octeocalcin (OC), carboxyterminal cross-linked telopeptide of type I collagen (CTx), latenight cortisol in serum were assayed by electrochemiluminescence (ECLIA). 24h urinary free cortisol (24hUFC) was measured by an immunochemiluminescence assay (extraction with diethyl ether). Functional assessment was performed using «chair rising», «up and go» and «tandem» tests. Universal pain assessment tool (verbal descriptor scale, Wong-Baker facial grimace scale, activity tolerance scale), EQ-5D and ECOS-16 questionnaires were given to patients and they self-reported their conditions. Results: Among 215 patients, 178 were females and 37 males, median age 35 (Q25-Q75 27-48); 88patients (40,9%) had low traumatic fractures, including vertebral fractures in 76 cases (in 60 cases multiple vertebral fractures) and non-vertebral fractures in 27 cases (17 patients had rib fractures, 3 -fractures of metatarsal bones, 2 fractures of radius, 2 fractures of tibia and fibula, 1 humerus, 1 breastbone; 1 hip fracture). Patients with fractures had higher 24hUFC, late-night cortisol in serum, lower OC, Total Hip BMD, but did not differ in age, BMI, CTx or etiology of CS. After applying the logistic regression analysis (adjusted for sex, age, BMI, BMD, OC), the main predictor of fractures was late-night serum cortisol level (p=0,001). Patients with late-night serum cortisol higher than 597 nmol/l were more likely to have low traumatic fractures (Odds ratio 2,86 (95%CI 1,55-5,28) p=0,001). Patients with fractures suffered from more pain and reported worse functional abilities. They had slightly worse results in «tandem» test, but did not differ in other functional tests, which assessed mainly muscle power. Conclusions: Patients with CS have very high risk of low traumatic fractures. The severity of hypercortisolemia is the best predictor of low traumatic fractures in patients with CS. Patients with fractures sufferedfrom more severe pain and because of this they restricted their daily activity even more than patients with CS without fractures. Consequently, patients with higher levels of late-night serum cortisol need earlier preventive treatment for osteoporosis.

This review of the literature has been dedicated to experimental and clinical studies of mechanism of action and efficacy of 1—34 amino acid fragment of parathyroid hormone — teriparatide as well as others contries experience of its prescribtion. Teriparatide is an osteoanabolic agent which stimulates bone formation by affecting bone modeling and by stimulating bone remodeling. The effects on modeling lead to increased bone formation whereas the effects on bone remodeling lead to increased bone turnover. Thus, in its mode of action teriparatide differs from all others medicines currently available to treat osteoporosis. Daily subcutaneous injections of teriparatide are proved to be effective to prevent low-traumatic vertebral and non-vertebral fractures in postmenopausal women with the history of vertebral fractures. Teriparatide is effective to treat osteoporosis in male and even more effective than alendronate to treat glucocorticoid-induced osteoporosis. Due to high cost and some restriction related to the duration of therapy (up to 18 months in Russia and 24 months in others countries) teriparatide should be recommended to treat severe osteoporosis in patients with a history >1 moderate clinical vertebral fracture or two or more vertebral fragility fractures or in case the previous treatment was not effective. Teriparatide should be prescribed after bisphosphonates or other antiosteoporotic treatment, but not in the combination with bisphosphonates. The prescribtion of bisphosphonates after teriparatide is effective to maintaine and further improve the effect. Thus, teriparatide is effective to treat severe osteoporosis and osteoporosis resistant to other therapy.

The article briefly reviews the specific features of target-organ lesions in multiple endocrine neoplasia type 1 (MEN1) syndrome and a clinical case of genetically confirmed MEN1 syndrome in a young female patient. Despite the relative rarity of this disease, timely diagnosis, treatment and screening for its main components are very important for the overall prognosis of patients with MEN1 and their first-degree relatives who are MEN1 gene mutation carriers. The described case is noteworthy for a number of specific features. The authors could find no account of optic chiasm glioma within the framework of MEN1 in the literature. Moreover, therapy-resistant somatoprolactinoma engages attention, which points to its aggressive nature with pituitary adenoma that is not been clearly visualized on magnetic resonance imaging. Of interest is the order of detection of neoplasms, in particular the manifestation of hypoglycemic episodes as a sign of organic hyperinsulinism. which have been initially regarded as epileptic seizures, after the use of sustained-release somatostatin analogues for the treatment of acromegaly.Представлены краткий обзор особенностей поражения органов-мишеней при синдроме множественных эндокринных неоплазий 1-го типа (МЭН-1) и клиническое наблюдение генетически подтвержденного синдрома МЭН-1 у молодой пациентки. Несмотря на относительную редкость этого заболевания, своевременная диагностика, лечение и скрининг основных его компонентов крайне важны для общего прогноза у пациентов с МЭН-1 и их родственников первой линии родства - носителей мутации в гене MEN1. Представленный случай примечателен рядом особенностей. В литературе мы не встретили описания глиомы хиазмы в рамках МЭН-1. Кроме того, обращает внимание резистентность соматопролактиномы к терапии, что указывает на ее агрессивный характер, в отсутствие четкой визуализации аденомы гипофиза по данным магнитно-резонансной томографии. Интересна и последовательность выявления новообразований, в частности проявление эпизодов гипогликемии как признака органического гиперинсулинизма, первоначально расцененных как эпилептические припадки, после назначения аналогов соматостатина длительного действия по поводу акромегалии.

Multiple endocrine neoplasia syndrome type 1 (MEN1, Wermer syndrome) – group o а heterogeneous inherited deseases, caused by hyperlasia or neoplasia of several endocrine glands. The phenotype of MEN1 is broad, and over 20 different combinations of endocrine and non-endocrine metabolic manifestations have been described. This case demonstrates multiple formations of endocrine organs, starting non-classical with macroprolactonoma resistant to dopamine agonists therapy, other endocrine disorders developed gradually eventually: hyperparathyreoidism and hypoglycemia caused by pancreas lesions, produced proinsulin in high levels.

The authors review the current literature data on the pathogenesis, clinical manifestations and treatment of glucocorticoid induced osteoporosis in patients with endogenous hypercortisolism. High levels of glucocorticoids lead to bone loss, bone quality deterioration and low traumatic fractures as a consequence of reduced bone formation. In addition to this, muscle weakness and sex steroids hormone abnormalities increase the risk of falls and fractures. The new available data on possible mechanisms of these changes including the involvement of RANKL/RANK/OPG, Wnt-betacatenin signaling pathway and pathogenesis of myopathy are discussed. This review also outlines the practical recommendation and new questions that should be evaluated in future research.

Purpose. Endogenous Cushing’s syndrome (CS), usually affecting young and otherwise healthy patients, is a good model to validate the effects of supraphysiological levels of glucocorticoids in humans. This study evaluates circulating levels of extracellular antagonists of Wnt/ß-catenin signaling pathway (sclerostin, Dickkopf1 (Dkkl), secretedfrizzled-related protein 1 (SFRP1)) along with osteoprotegerin (OPG) and soluble receptor activator of nuclear factor kappa-beta ligand (RANKL) in patients with CS as compared to healthy individuals. Materials and methods. Forty patients with clinically and biochemically evident CS and 40 sex, age and body-mass index matched healthy individuals provided fasting serum samples (8:00-10:00AM) for measurement of sclerostin, SFRP1 and Dkkl, RANKL., OPG along with bone turnover markers. Serum samples on RANKL., OPG., Dkkl, SFRP1, sclerostin were frozen and then concurrently measured by an enzyme immunoassay (ELISA) using commercially available reagents. Serum samples on osteocalcin (OC), carboxyterminal cross-linked telopeptide of type I collagen (CTx), cortisol in serum and saliva were assayed by electrochemiluminescence (ECLIA) Cobas e601 Roche. Urinary free cortisol (24hUFC) was measured by an immunochemiluminescence assay (extraction with diethyl ether) on a Vitros ECi. All participants were questioned regarding any recent low traumatic fractures. Patients with CS underwent standard spinal radiographs in anterior-posterior and lateral positions of the vertebrae Th4-L4 (Axiom Icons R200 "Siemens"). Results. Patents with CS (30 (26-40) years old with 24hUFC 2575 (1184-4228) nmol/l (Me (Q25-Q75)) had suppressed OC and normal CTx levels as compared to healthy subjects. A significant correlation, which we observed between OC and CTx (po=0.724 (p

A 53-year old man with acromegaly was referred for neurosurgical treatment. At admission: growth hormone (GH) 240 ME/l at all time points of glucose tolerance test, IGF-1 - 415,1 ng/ml, brain MRI showed pituitary adenoma 34×32×27 mm extending suprasellar, deforming and displacing optic chiasm, parasellar to both cavernous sinuses and surrounding left internal carotid artery. Clinical blood tests revealed marked erythrocytosis - 6.1×1012, Hb - 200 g/l, HCT - 62%; urobilinigen 50 mmol/l was found in urine; otherwise the tests were normal. For exclusion of erythraemia the patient underwent trepanobiopsy of bone marrow which showed normal haemopoesis and suggested a secondary nature of erythrocytosis. In preoperative period 1100 ml of blood were evacuated by phlebotomy in 4 sessions, which resulted in reduction of erythrocyte count to 5,1×1012, Hb 160 g/l, HCT 50% and disappearance of urobilinigen. The patient was then operated by transnasal approach with debulking of most adenomatous tissue but without biochemical remission of acromegaly (GH 92-99 at glucose tolerance test). A week after surgery - erythrocytes 4,5×1012, Hb - 141 g/l, HCT - 44,1%. The patient was prescribed a octreotide depo (Sandostatin LAR) injections 20 mg/mth and at 6 months from operation was feeling well, with clinical remission of acromegaly and normalization of IGF-1 and all blood count parameters.

The main manifestations of central diabetes insipidus (CDI), a disease of absolute deficiency of hypothalamic hormone vasopressin, are severe thirst and polyuria, which severely interferes with normal life of patients. In some cases CDI may be the first sign of wide spread metastatic process, appearing many years after successful treatment of oncological diseases.

Calcium-sensing receptor (CaSR), a member of G proteincoupled receptors family, plays a key role in systemic calcium homeostasis regulation. It is expressed in a variety of tissues, and is responsible for maintaining plasma calcium concentration. In addition, the CaSR is known to regulate some important cellular processes such as apoptosis and proliferation. CaSR is a drug target for calcimimetics and calcilytics, agents used for modification of parathyroid cells function and treatment of some pathologic conditions. Inactivating or activating mutations of the CaSR gene cause different disorders of calcium metabolism. Polymorphic variants of the gene have been described in healthy populations.

Primary hyperparathyroidism (PHPT) is the third most frequent endocrine disorder and has a variable clinical presentation. Asymptomatic PHPT became the predominant form of the disease with increase of its incidence after the introduction of automated serum calcium measurement in North America and Europe. Data from Russia is lacking. Aim: To present the clinical profile of PHPT in Russia. Materials and Methods: This retrospective study was conducted at endocrinology centers in 8 regions of Russia. We analyzed the clinical presentation, and treatment options in patients with confirmed PHPT (1995-2010). Results: 738 patients (F: M-8:1) with age ranging from 13 to 83,4 years (mean 54,3) were analyzed. 54% was from Moscow (n=397), 11% - from Moscow region (n=79) and 35% - from 53 regions of Russia (n=262). Symptomatic PHPT was the most common form (74%) and was revealed with osteoporosis in 56%, nephrolithiasis - in 45% and ulcer disease - in 18%. Our data showed an increase in the incidence of PHPT (especially mild PHPT) after 2005 compared with earlier period. 64,9% of patients were treated surgically, 17 patients undergone repeated parathyroid surgery. 28,3% received bisphosphonates, calcitonin and/or cinacalcet . 11,5% was observed without treatment. Conclusions: This data analyzes some causes of delayed diagnosis of PHPT in Russia, characterized age and gender distribution of patients with PHPT and demonstrates the changes in clinical profile of disease from 1995 to 2010. PHPT still remains symptomatic disorder in our country most frequently with skeletal and renal manifestations.

In this article we describe a clinical case of primary hyperparathyroidism, gout tophus and diabetes mellitus type 2. The relationship between hyperuricemia and hypercalcemia linked to primary hyperparathyroidism is discussed.

Primary hyperparathyroidism (PHPT) is a common endocrine disorder that affects bones, urinary tract, cardiovascular system, gastrointestinal tract, and another organs and tissues. The most cases of PHPT are mild forms and those patients have nonspecific symptoms, which require careful diagnostic considerations and choice of treatment. There is not enough data about prevalence of PHPT, especially it's mild forms, in Russian Federation, and future epidemiological research needs to be done. Present literature review includes information about etiology, pathogenesis, clinical findings, diagnostic and differential diagnostic, indication for surgery and medical treatment of PHPT. The review is based on data of studies from the different countries and few studies from Endocrine science center.

Despite the relatively low incidence of acromegaly (60-70 cases per I million inhabitants), this disease has a special place among the heterogeneous group of diseases that lead to the defeat of the locomotor apparatus. The slow growth of the clinical manifestations of acromegaly and as a consequence, late diagnosis, the cause of early disability and premature death of patients. In order to improve the quality of life and social adaptation of patients to date is an obvious need to identify groups of patients with acromegaly, requiring additional therapy for osteoporosis and osteoarthritis. We performed the search in bibliographic bases MEDLINE and Cochrane Collaboration from 2000 on 2009. Key words were the following: acromegaly, acromegaly and arthropathy, osteoporosis and acromegaly, the bone mineral density and acromegaly, fractures and acromegaly. In this article the data about role of risk factors for the defeat ofosteoarticular apparatus, the dynamics of the articular syndrome and the state of bone tissue in acromegaly.

Primary hyperparathyroidism (PHPT) is a common disorder often associated with reduced bone mineral density (BMD) and osteoporosis. The main goal in treating patients who refuse surgery is the correction of bone complications. The aim of this study is to evaluate safety and efficacy of alendronate treatment in patients with PHPT and osteoporosis. Eligible patients were postmenopausal women suffering from PHPT who did not meet surgical guidelines or ignored surgery due to different reasons. 55 osteoporotic or osteopenic women received alendronate 70 mg weekly and 43 were leaved under medical observation for the reasons of not being osteoporotic or financial unable to comply with treatment/ Results: After 2 years of treatment alendronate group showed statistically significant gain in BMD at L24 +4,0±4,2%, Neck +2,2±4,4%„ TH +2,3±4,5%. At the same time observational group showed decrease in BMD at L24 -4,2±4,0%, TH -3,5±3,8%, R33% -4,0±6,5%, [95% CI -7,6; -0,4]. On the whole BMD dynamics in groups differed at the following sites: L2 (p=0,00004), Neck (p=0,005), TH (p=0,001), R33% (p=0,006). ' Alendronate intake was accompanied by serum Ca decrease (at 1 year point -4,3±5,4%, p=0,00005, at 2 year -2,7±6,4%, p=0,04), 24 hours'Ca excretion decrease from (Me) 8,5 to 6,3 mmol/day at 1 year point, (p=0,007) without PTH elevation. As well as CTx fell at Me: 66% at 1 year and at 60% at 2 year point and OK at 39% and at 27%, correspondingly, p

The reported clinical case represents an example of characteristic traits in lipid metabolism and fat tissue deposition in hypercorticosolism. Probable causes for fat mass expansion in glucocorticosteroid excess, its distinctive cushingoid distribution, role of 11β-HSD1 and glucocorticoid receptors and reversibility of specific abnormalities after correction of hypercortisolism are discussed in the context of physiological and pathological aspects of glucocorticosteroid action.

This paper reports two clinical cases of ACTH-dependent hypercorticism. Difficulties encountered in differential diagnosis of this condition were due to poor informative value of routine diagnostic methods. The use of selective blood collection from the inferior petrosal sinuses (for the first time in Russia) greatly facilitated the choice of the adequate treatment strategy. A detailed description of clinical features of ACTH-dependent hypercorticism in two young women is presented along with the results of laboratory and instrumental studies. Technical aspects of selective blood collection from the inferior petrosal sinuses in conjunction with the desmopressin stimulation protocol are considered. Indications for the use of this diagnostic method under real clinical conditions are proposed. Results of the study are discussed with reference to the treatment strategy chosen for the management of ACTH-dependent hypercorticism and the outcome of surgical intervention.

The cardiovascular system was analyzed in patients with symptomatic (n = 31) and mild primary hyperparathyroidism (n = 34) whose mean age was 54.6 years; 95% females). In the patients with symptomatic primary hyperparathyroidism, the PQ interval was longer and the QT interval was significantly shorter than those in patients with mild hyperparathyroidism. Left ventricular hypertrophy was noted in 45.2% of patients with symptomatic and in 15.2% of those with mild hyperparathyroidism (p = 0.013). Left ventricular diastolic dysfunction was also more common in the group of symptomatic hyperparathyroidism. There was a statistically significant correlation between the levels of parathyroid hormone, total and ionized calcium and the duration of QT interval and the determinants of diastolic function and left ventricular hypertrophy. The revealed cardiovascular disorders in patients with primary hyperparathyroidism are presumed to depend on the increase rate of parathyroid hormone and total and ionized calcium.

Hypogonadism is the most popular cause of BMD decreasing not only in women, but also in men. There are several studies showed a correlation between duration of hypogonadism and BMD reducing degree. Despite wide light exposure of osteoporosis problem at women with hyperprolactinemia, there are a few researches devoted to the same question at men. The aim of our study was to assess the biochemical parameters of bone metabolism and the bone mineral density (BMD) in men with tumoral and not tumoral hyperprolactinemia. In 24 men with hyperprolactinemia and 17 healthy controls we evaluated BMD, serum osteocalcin (OK) and C-terminal telopeptide of type I collagen (CTX), as well as calcium total, ionized calcium and alkaline phosphatase. All men with hyperprolactinemia were also studied after 24 months of cabergoline treatment. The results showed significant differences between study groups in BMD of vertebra and femur bone (p = 0,000052 up = 0,002718). We confirm increasing of BMD in vertebra and femur bone after cabergoline treatment (p = 0,003 и p = 0,027).

The aim was to estimate the effects of treatment with alendronate (Fosomax 35 mg) in postmenopausal women with osteoporosis and subclinical hyperthyroidism. Thirty postmenopausal women (64 (60-69) years old) with osteoporosis (T-score ≤ -2,5) and subclinical hyperthyroidism (77% with endogenous subclinical hyperthyroidism and 23% on L-thyroxine suppres-sive therapy after thyroidectomy due to differentiated thyroid cancer) were randomly assigned into two groups: 1-14 women received Fosamax 35 mg a week in combination with 500 mg of calcium and 400 UI of Vitamin D3 (VD) daily; 2-16 women received 1000 mg of calcium and 800 UI of VD daily. Euthyroidism was achieved in all women with endogenous subclinical hyperthyroidism. An increase in physical activity was recommended to all patients and a hypolipidemic diet was given to those who had had high cholesterol level. Biochemical parameters (calcium (Ca), phosphorous (P), creatinine (Cre), alkaline phosphatase (ALP), cholesterol, low density lipoprotein (LDL), high density lipoprotein (HDL), triglycerides (TG), cholesterol/HDL ratio) in fasting serum as well as calcium/creatinine ratio in fasting urine (U-Ca/U-Cre); biochemical markers of bone metabolism: osteocalcin (OC) and C-terminal telopeptide of type I collagen (b-CTx) serum ("ECLIA", Roche Elecsys 1010/2010), BMD (DXA; Prodigy, Lunar) at the lumbar spine (L1-L4), femoral neck (FN), total hip (TH) and radius total (RT) were measured at the baseline visit and after 1 year of treatment. At the baseline visit there were not found any differences between the 1 and the 2 groups. After 12 months of treatment the markers of bone metabolism as well as ALP decreased significantly in both groups, though the decreases were significantly greater (p

For many years, hyperparathyroidism, including primary, primarily associated with severe pathology of the osseous system and kidneys, was considered a rare disease. The widespread introduction into the clinical practice of the determination in the blood of calcium, and then parathyroid hormone, and osteodensitometry made it possible to recognize this disease more often and at earlier stages and to treat it more successfully. By now, the specific gravity of mild and asymptomatic forms of hyperparathyroidism has increased from 10-15% in the 1980s to 80%. Conservative management of these forms of hyperparathyroidism requires more thorough research on the prognosis of survival, duration and quality of life, and the risk of developing associated diseases in these patients.

The aims of the reported multinational open study were to estimate the influence of subcutaneous injections of 20 mkg daily Teriparatide on bone mineral density (BMD) at the lumbar spine and proximal femur, the markers of bone metabolism, the quality of life as well as the safety of treatment. Duration of the treatment period was one year. Fifty postmenopausal women with osteoporosis (L1-L4 T-score mean -3,2±0,6 SD) were enrolled in the study. 70,8% of patients had fragility fractures. Forty four patients (78%) remained until the end of the study. BMD was detected by dual energy X-ray absorptiometry (DXA) («Prodigy», Lunar and «Hologic» 4500 W) at the lumbar spine (L1-L4) and proximal femur with converting to the standardized BMD. Bone alkaline phosphatase (BAP) and osteocalcin (OC) were measured as markers of bone formation and C-terminal cross-linking telopeptide (CTX) were measured in serum as a marker of bone resorption. The significant increase in BMD was found at the lumbar spine (L1-L412,5±6,6%), whereas BMD at the proximal femur did not change significantly. The levels of markers of bone metabolism were increased after 3 months of the treatment and to the end of the study were 192,2%; 385%; 526,3% higher versus the baseline levels for BAP, OC., CTX consequently. There was also found a significant improvement in the quality of life of the patients which was estimated by EQ-5D inquirer at the baseline visit and at the end of the study. The adverse effects, which were related to the teriparatide injections, were observed in 44% of the patients. Due to the adverse effects Tereiparatide was cancelled for 6 (12%) patients. In other cases the adverse effects were slight and the cancellation of treatment was not required. Conclusions: The daily injections of Teriparatide (20 mkg) during 1 year provide the significant increase in BMD at the lumbar spine for postmenopausal women with osteoporosis as well as the early increase in the bone turnover rate. Treatment with Teriparatide improves the quality of life for women with postmenopausal osteoporosis. The daily injections of Teriparatide during 1 year are well tolerated and safe.

An open clinical trial evaluating effects of Pravastatin (P) 20 mg, Fluvastatin (F) 40 mg versus Calcium-D3 Nycomed Forte (1000 mg calcium and 800 ME cholecalciferol - CaD3 ) on bone metabolism, bone mineral density (BMD) and lipid metabolism have been conducted. We studied 80 osteopenic women from 60 to 80 years old, divided into three groups: 30 participants were treated by CaD3 during 12 months, at the same time 30 ones applied P, and 20 women were treated by L for the 6 months. All of the remedies showed similar influence on the spine BMD (the basal BMD remained intact). CaD3 and L were able to prevent proximal hip from BMD loss whereas patients treated P proceeded to lose bone mass (up to 1.3-1.5%). We have found that CaD3 provides an antiresorptive action (СТх became 27.2% lower and OC -4.1%), that P doesn't touch bone metabolism indices and dual action of L: it suppress a bone resorption (СТх was 14,1% reduce) and show an invigorative action on bone formation (ОК 25,5% raised). The CaD3 treatment was the less expensive, and the highest cost had P course. Our comparative study reveals that CaD3 is preferable in solitary osteoporosis prevention. However when osteopenia or validity OP risk factors coexist with dyslipidemia, type II particularly, use of L may be rational because of it's 6th month therapy had rather equal effect to one of year CaD3 consumption.

The purpose of the study was to define indications for surgical treatment and its scope in patients with secondary hyperparathyroidism in the presence of end-stage chronic renalfailure. The authors examined 80 patients who had a history of long-term procedures of hemo- or peritoneal dialysis. The patients’ mean age was 47±3.2 years. Measurements of the levels of alkaline phosphatase, ionized and total calcium, phosphorus, parathyroid hormone, ultrasound of the parathyroid glands, densitometry and X-ray study were made in all the patients. All the patients received alfacalcidole therapy during different periods of time. Clinical, laboratory, and morphological correlations were made to establish indications for surgical treatment. The sensitivity ofpreoperative ultrasonography was 72.5% and that of intraoperative ultrasound study was as high as 98.4%. The sensitivity of intraoperative revision was 75%. Sixteen of the 20 patients operated on underwent total parathyroidectomy by autografting a fragment of one of the least glands into the muscle. Subtotal parathyroidectomy was made in 3 patients; 3 parathyroid glands were removed in 1. Emergency and planned studies were performed in all the patients. The duration of the patients operated on averaged 14 months. Three patients undergone total parathyroidectomy with autografiing developed signs of hyperparathyroidism following 6, 12, and 13 months, in this connection graft resection was made in these patients. In the follow-up periods of 3 to 6 months, the level of parathyroid hormone became normal after resurgery in 2 of these patients, hypoparathyroidism developed in one patient. Two months after surgery, recurrent secondary hyperthyroidism was detected in 2 of the 3 patients who had undergone subtotal parathyroidectomy (4 and 7 months after the occurrence of signs of transient hypoparathyroidism) and in 1 patient in whom 3 parathyroid glands had been removed. Four of the 20 patients operated on were observed to have hypoparathyroidism that was compensated by calcium preparations and active forms of vitamin D3. Thus, a good result was noted in 70% of the patients after surgical treatment.

The article devoted to the secondary hyperparathyroidism in patients with end-stage chronic renal failure

The article is devoted to the study of the complete form of the testicular feminization syndrome with dysherminoma in one testicle combiner with prolactinoma.

Schwartz-Bartter Syndrome (synonyms: syndrome of inappropriate secretion of antidiuretic hormone syndrome of inappropriate secretion of vasopressin, anti-diabetes insipidus) - this is a rare disease characterized by excessive secretion of antidiuretic hormone (ADH) from the posterior pituitary, or other source, resulting in developing hyponatremia and water intoxication. Hyponatremia dilutions at Schwartz-Bartter Syndrome is usually non-specific symptoms, such as headache, nausea, vomiting and confusion. Marked reduction in blood sodium levels may lead to seizures or even coma.

Three groups of premenopausal women with diffuse toxic goiter (DTG) of different duration and different activity of thyrotoxicosis were examined for assessing calcium-phosphorus metabolism, osseous metabolism, and mineral compactness of bone tissue by ultrasonic (US) densitometry and bienergetic x-ray absorptiometry. Manifest hypercalciuria and hyperphosphaturia with normal levels of Ca2+ and inorganic phosphorus in the blood were revealed in patients with newly detected and recurrent DTG. In patients with hyperthyrosis the blood alkaline phosphatase activity and hydroxyproline excretion with the urine on an empty stomach were increased, these changes being most of all expressed in the group with newly detected thyrotoxicosis; however, these shifts were liable to normalize when stable drug-induced euthyrosis was attained. A reliable correlation between free T4 in the blood serum and osseous metabolism was revealed, which may indicate a direct effect of TH excess on bone tissue metabolism. The osteopenic syndrome was detected primarily in the proximal portions of the femoral bone, the severity of osteopenia reliably correlating with the disease duration. The authors come to a conclusion about the informative value of US densitometry and bienergy x-ray absorptiometry for the diagnosis of early stages of the osteopenic syndrome in DTG.

Background. Secondary hyperparathyroidism (sHPT) is one of the serious complications in chronic kidney disease and is associated with progressive bone disease and vascular calcification.The objective of the study was to determine the impact of Mimpara (Cinacalcet HCl) on mineral disorder, bone turnover and bone mineral density (BMD) versus parathyroidectomy (PTx) in haemodialysis patients’ refractory to alfacalcidol. Materials and methods. 62 haemodialysis patients with sHPT were enrolled in this 6=months prospective study. All of them had surgical indications for PTx. Surgical indications was established according to clinical or biological assessment. 40 patients underwent Mimpara treatment. Dose of Mimpara was titrated every 4 weeks. Sequential doses included 30–180 (mean 59.1 ± 34.2) mg/day. 22 patients underwent PTx. The surgical technique was depended on quantity of hyperplastic parathyroid glands.Results. In 6 months mean iPTH, Ca, Са×Р, CTx and OC levels significantly decreased by 55.7%, 13.8%,34.3%, 21.4 and 1.4% in the Mimpara group vs. 90.7%, 14%, 55.5%, 58.7% and 26.9% in the PTx group. Median serum iPTH level decreased by 30% after initiation of Mimpara in 94.3% patients, from them by 50%in 74.3%. Achieved the KDOQI treatment targets for PTH in 28.6% patients.In 6 months after PTx median serum iPTH level was