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The topical diagnosis of insulinoma continues to be an actual problem of modern medicine due to low detection rate of the tumor (about 75%) and inconsistent data on its localization when using various methods of visualization (more than in 50% of cases), as well as the lack of a single diagnostic algorithm. In the clinical practice, many different imaging assessments are conducted, including those with the administration of contrast agents and associated with radiation load, as well as invasive studies, associated with risk of various complications, high costs and duration of the examination. Thus, the search for highly sensitive and safe methods of topical diagnosis of the insulin-producing pancreatic tumor seems relevant. In the presented clinical case, the diagnosis of insulinoma is verified by diffusion-weighted magnetic resonance imaging (DW-MRI). It is an accurate and non-invasive method that does not involve exposure to ionizing radiation and does not require any administration of contrast agents. This technology, based on microstructural pathological changes, allows to identify small tumors and to make a differential diagnosis of benign and malignant neoplasms. However, at present the experience of DW-MRI use for the diagnosis of an insulin-producing neuroendocrine tumor is limited. Evaluation of its effectiveness in a large patient cohort would be necessary to assess the prospects for its introduction into clinical practice.

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

Insulinoma is the most common functioning tumor of the pancreas. It consists of β-cells of the Langerhans islets. Insulinoma is malignant in 10% of cases and multifocal in 10% of cases. This review discusses the new capabilities for identification of the exact location of insulinoma, in particular given the latest data on expression of different receptors in the tumor tissue. We discuss the modern concept of molecular and genetic aspects of insulinoma development. In particular, we analyze studies on germinal and somatic mutations and epigenetic abnormalities. We describe new biochemical markers of insulinoma, which may be used for differential diagnosis, timely detection of insulinoma recurrence and progression, individual prognosis, and monitoring of treatment. Also, we discuss application of antitumor medications and optimal drug correction of hypoglycemia in insulinoma patients.

Отделение детской хирургии на базе ФГБУ “Эндокринологический научный центр” функционирует около двух лет. За время работы хирургическое лечение получили более 500 пациентов с различной эндокринной патологией. В статье обсуждаются современные диагностические подходы и выбор хирургической тактики при заболеваниях, входящих в круг интересов нового направления детской хирургии – эндокринной хирургии детского возраста. В рамках дискуссии обсуждаются варианты радикального лечения болезни Грейвса у детей, рассматриваются положительные и отрицательные стороны хирургического лечения и радиойодтерапии. Приводится собственная статистика послеоперационного гипопаратиреоза. Предлагается оптимизация алгоритма действий при выявлении узлового зоба у детей. При первичном гиперпаратиреозе делается акцент на сложностях послеоперационного ведения пациентов, связанных с особенностями детского возраста, определяющими остроту реакции на водно-электролитные нарушения. Отдельно рассмотрены данные литературы по заболеваниям надпочечников у детей, демонстрируются собственные клинические случаи, потребовавшие хирургического вмешательства. Освещаются возможности современной нейрохирургической техники, применяемой в Эндокринологическом научном центре при операциях на гипофизе у детей. Пациентам различных возрастных групп проводится трансназальное транссфеноидальное удаление опухолей хиазмально-селлярной области с применением эндоскопической ассистенции. В статье также приведены данные исследований в области изучения болезней поджелудочной железы и их хирургического лечения. Большое внимание уделяется гендерному разделу эндокринной хирургии детского возраста. Обсуждается тактика при нарушениях формирования пола, опухолях половых желез у детей, заболеваниях молочных желез. В заключение обрисовываются перспективы развития эндокринной хирургии детского возраста.

В представленных клинических рекомендациях обсуждаются современные подходы к диагностике и лечению дифференцированного рака щитовидной железы у взрослых. Изменения в настоящей редакции Клинических рекомендаций касаются показаний к пункционной биопсии, скринингового определения концентрации кальцитонина, унификации заключений цитологического исследования, новых подходах к послеоперационной динамической стратификации риска рецидива, показаний к супрессивной и заместительной терапии, таргетной терапии йоднегативных форм дифференцированного рака щитовидной железы.

Обсуждается диагностический алгоритм при случайно выявленных образованиях надпочечников, направленный на определение гормональной активности и злокачественного потенциала, показания и ранжированность наблюдения для группы пациентов с гормонально неактивными доброкачественными опухолями. Рекомендовано определение гормональной активности образования, которая может проявляться гиперкатехоламинемией, эндогенным гиперкортицизмом, первичным гиперальдостеронизмом. Всем пациентам рекомендовано определение кортизола в утренние часы на фоне подавляющего теста с 1 мг дексаметазона. При отсутствии физиологического подавления уровня кортизола в качестве подтверждающего теста предлагается использовать определение адренокортикотропного гормона в утренние часы. В качестве метода первичной диагностики феохромоцитомы/параганглиомы (ФХЦ/ПГ) рекомендовано определение свободных метанефринов плазмы или фракционированных метанефринов суточной мочи. При положительном результате тестирования необходимо углубленное обследование для исключения или подтверждения ФХЦ/ПГ. При наличии у больного с опухолью надпочечника артериальной гипертензии рекомендовано определение соотношения между уровнем альдостерона и активностью ренина плазмы (прямой концентрации ренина) для исключения первичного гиперальдостеронизма. Для диагностики злокачественного потенциала опухоли рекомендована оценка количественных денситометрических показателей компьютерной томографии. Лечебная тактика в отношении инциденталом надпочечников не рассматривается в настоящих рекомендациях, так как является сферой компетенции соответствующих рекомендаций по конкретным нозологиям.

When primary hyperparathyroidism (PHPT) is diagnosed in a young patient in the absence of other clinical manifestations differential diagnosis between a sporadic form of PHPT and PHPT as the first manifestation of one of hereditary syndromes may be challenging. Diagnosis of sporadic or hereditary PHPT determines the extent of surgical intervention, a strategy for further observation and treatment, and the need for examination and treatment of first-degree relatives. Aim of the study — to determine genetic characteristics of PHPT with manifestation at a young age (A. In 4 (4/40, 10%) patients we revealed germline heterozygous mutations in CDC73 gene: 3 nonsense mutations in patients with parathyroid carcinoma — in exon 3 p.R91X, exon 6 p.Q166X, exon 7 p.R229X, and 1 missense mutation in a patient with parathyroid hyperplasia in exon 8 p.R263C. Conclusions. In the majority of cases (75%) PHPT in young patients without positive family history is sporadic. Search for germline mutations in the genes, leading to development of hereditary forms of PHPT (first of all in MEN1 and CDC73), is appropriate in young patients with positive family history, or when positive family history may be suspected, and in patients with parathyroid carcinoma. In the majority (75%) of FIHP cases search for other, probably yet unknown, genes is necessary.

Subclinical Cushing’s syndrome is the most widespread variant of hormonal activity among adrenal incidentalomas. The clinical significance of this condition has been confirmed by numerous studies showing “metabolic consequences” of chronic autonomous cortisol hypersecretion. Some clinical symptomatology of metabolic syndrome, such as obesity, impaired carbohydrate metabolism and hypertension, are peculiar to subclinical hypercorticism more than to population. This requires a thorough examination of patients with adrenal incidentalomas for hormonal activity, as well as determining the indications for surgical intervention. In the current era of evidence-based medicine, the overall management of these tumors remains empirical. The pathogenesis of subclinical Cushing’s syndrome remains unclear. Understanding of the mechanisms of chronic autonomous cortisol hypersecretion would enable to identify clinical and genetic heterogeneity of this condition, to determine prognosis and indications for surgery and to develop pharmacological methods of treatment. The purpose of this article is to review the current views on the diagnosis, pathogenesis and treatment of subclinical Cushing’s syndrome, identifying perspective directions of work.

Russian clinical practice guidelines are dedicated to the management of patients with pheochromocytoma and paraganglioma.

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.

Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.

Objectives. T study the quality of life and status of carbohydrate metabolism in patients after surgical treatment insulinoma. Methods: The study involved 20 patients divided in two groups: the first group with a catamnesis duration of up to five years; the second group with a catamnesis duration of more than five years. We studied anthropometric parameters and carbohydrate metabolism as well as psychological questioning of patients using SF-36 questionnaire, the data was considered statistically significant at p0.05. Results. severe combined postoperative complications were more frequent in the first group (63.6% vs. 22.2%, p=0.07), due to extend of the performed surgery. Adrenergic symptoms prior to the surgery were detected in 90.9% of cases in the first group and in 77.7% of cases in the second group. After treatment these numbers decreased to 36.4% and 11.1% respectively (p=0.039 and 0.026). Neuroglycopeniс symptoms before treatment were detected in 90.9% of cases in the first group and for all patients in the second, while after treatment persisted only in 45.5% and 33.3% of cases respectively (p=0.045 and 0.036). Carbohydrate metabolism have normalized for the majority of patients. Two patients (18.2%) of the first group showed impaired glucose tolerance. Improved carbohydrate metabolism was associated with a decrease in body weight in both groups. Results of psychological questionnaires were comparable with the survey data obtained in general population in the Russian Federation. Conclusion. Surgical treatment of insulinomas is highly effective. Physical and psychological status of patients in most cases corresponds with those typical for this age-sex group of the population of the Russian Federation. Long-term treatment results do not depend on duration of the catamnesis. Complications that developed from surgical treatment have the main influence on the health of patients.

The aim of this prospective study was to determine the main factors influencing the development of postoperative hypocalcemia in patients with Gravesdisease after thyroidectomy. Assess whether patients with BG likely to develop postoperative hypocalcemia than those who performed thyroidectomy about nontoxiс multinodular goiter. In this study were prospectively included 54 patients with Gravesdisease, which from October 2011 to May 2013 was performed thyroidectomy. Additionally, as a control group from the database included 48 patients with nontoxiс multinodular goiter, who underwent thyroidectomy in the same time. All patients attended deficit/insufficiency of vitamin D (25(OH)D were 20/30 ng/ml). Patients were divided into 2 groups according to the postoperative calcium concentrations corrected for albumin in the blood: Group 1 - patients with postoperative levels of calcium in the blood of 2.0 mmol/l or less; Group 2 - Patients with blood calcium levels above 2.0 mmol/l. Thus, when patients Gravesdisease Group 1 disease duration was significantly higher concentration of 25(OH)D and postoperative parathyroid hormone was significantly lower compared to the 2nd group. According to logistic regression analysis, postoperative PTH level below 10 pg/ml was the main predictors of postoperative hypocalcemia ( p < 0,001). Patients with Gravesdisease increasingly requiring the appointment of calcium after the operation, they are demonstrating a significant clinical signs of hypocalcemia than patients with nontoxiс multinodular goiter after thyroidectomy.

Postoperative hypocalcemia is the most frequent complication after thyroidectomy. One goal of our study was to investigate the parathyroid hormone (PTH) as the main predictor and early postoperative hypocalcemia. The study prospectively included 135 patients who from October 2011 to May 2013 was performed thyroidectomy. According to the results according to logistic regression analysis, postoperative PTH level below 10 pg/ml was the main predictor of postoperative hypocalcemia ( p

Statement of hormonal activity and potential malignancy are the most important issues of diagnosis and selection of appropriate treatment way for patients with adrenal tumors. Adrenal cortical carcinoma (ACC) is rare disease with poor prognosis. Incidience of ACC is about 0.5-2 per million, and no more then 2-4% among adrenal tumors. This draft of guidelines of diagnosis and treatment of ACC is offered to common discussion among wide round of specialists.

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

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

The results of analysis of mortality and probability of the development of "uncontrollable hemodynamics" syndrome were used to estimate the necessity of preoperative preparation for the patients with pheochromocytoma to be exposed to different treatment strategies at different time periods. The scheme of preoperative preparation described in this paper is based on the experience with the application of two different regimens of preoperative prescription of alpha-adrenoblockers to 106 patients.

Topical diagnostic methods in ACTH-producing neuroendocrine tumors with various localization. Topical diagnostic of ACTH-producing neuroendocrine tumors is a major stage in management of patients with ACTH-producing syndrome, because one of the main methods of treatment of such patients is surgical. There are several methods of topical diagnostic of ACTH-producing tumors with ectopic ocalization which have some advantages and disadvantages. At the same time until now any of existing methods of research doesn't guarantee 100% result that leads to obtaining the false positive conclusions and, as a result, carrying out unreasonable surgical operation. This report is about the main methods of topical diagnosis and their comparative characteristics. Besides possible ways to increase sensitivity and efficiency of methods and improve results are discussed in article.

Currently the “gold standard” in diagnostics of thyroid tumors is a fine-needle aspiration cytology (FNAC). However, FNAC cannot discriminate between benign and malignant thyroid tumors in 15 to 30% of observations. In order to develop an additional tool for differential diagnostics of thyroid tumors we evaluated the diagnostic performance of 3-antigen serum autoantibody signature in groups of benign ( n = 22) and malignant ( n = 26) thyroid tumors using a dot-blot ELISA-based analysis The sensitivity and specificity of resultant array were estimated to be 55–60% and 95–100%, respectively ( p < 0.001 according to one-sided Fisher Exact Test). Thus, we created a prototype antigen array for differential diagnostics of thyroid tumors which can be regarded as a platform for design of more complicated panel, highly sensitive in thyroid cancer detection, which can significantly improve the accuracy of preoperative diagnosis of thyroid cancer.

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

Diagnosis and treatment of ectopic ACTH-syndrome currently is one of the most challenging problems among other forms of endogenous hypercorticism. This syndrome is associated with presence of extrapituitary tumors characterized with different histogenesis and localization, which produce adrenocorticotropic hormone (ACTH), or – rarely – corticotrophin-releasing hormone. In most cases the ectopic synthesis of ACTH is performed in bronchial carcinoid tumors (36–46%), oat cell cancer (18–20%), medullary thyroid cancer (3–7%), pheochromocytoma (9–23%), other sites are infrequent (pancreas, thymus, parotid gland, ovaries, uterus, prostate, colon, stomach, esophagus, etc.). Much of these tumors are aggressive and are characterized with propensity to metastasize and relapse. Currently there are few contradictory data on the comparative evaluation of the effectiveness of methods of topical diagnosis of the source of ectopic ACTH-secretion, and therefore there is an urgent need to develop an optimal and most efficient algorithm for diagnostic procedures to determine the extent of the tumor in patients with ectopic ACTH-syndrome. Indications for surgery, timing and extent of surgical intervention, the effectiveness of the operation, the causes and frequency of relapses are still discussed.The present difficulties of diagnosis, as well as the lack of a unified approach to the treatment of this disease in the complex, often lead to the progression and development of a large number of serious complications functions of up to disability, which in turn does not lead to significant improvement of quality of life. Thus further research is necessary to study of this disease

We have analysed the results of radiological investigations (USI, CT, and MRT) involving 177 patients given surgical treatment at the Endocrinological Research Centre during 2006-2010. In addition, the study included measurement of cortisol levels in the dexamethasone suppression test, fractionated metanephrins, and the relationship between aldosterone levels and plasma rennin activity (in the patients presenting with arterial hypertension). Twenty two patients underwent tumour puncture. Seventy six (55.9%) of the 136 patients having incidentalomas were found to have no indications for the surgical intervention (because of the absence of hormonal activity and low malignancy potential of the neoplasm). The follow-up period averaged 24.9±19 months. The choice of indications for the surgical treatment should be based on the results of standardized hormonal studies and the determination of malignancy potential using the native CT density above 20 UH as the principal criterion. Differential diagnostics by USI, MRT, and descriptive CT is believed to be inadequate. USI may be applied as a highly specific method for the detection of cysts and myelolopomas. Native CT density should be taken into consideration for the interpretation of the results of fractionated metanephrin measurements. Diagnosis of pheochromocytoma is very unlikely in the patients presenting with low CT density. Puncture biopsy is indicated only when the presence of metastases in the adrenal glands is suspected.

Carcinoid syndrome develops as a sequel of enhanced secretion of vasoactive substances from neuroendocrine tumours (NET). Somatostatinoma is an extremely rare variant of NET that manifests itself in cholelithiasis, steatorea, and diabetes mellitus. Somatostatinoma is usually diagnosed in adult subjects. Carcinoid syndrome is not a typical consequence of somatostatinoma. We present the first case report of somatostatinoma that was diagnosed in a child having carcinoid syndrome. The diagnosis was made difficult by the negative results of the search for biochemical markers of carcinoid syndrome and the small size of the primary tumour that could not be visualized by computed tomography. The immunohistochemical studies demonstrated secretion of somatostatin and calcitonin. The treatment of the child with somatostatin analogs proved inefficient which can be accounted for by the late diagnosis of the disease and the extensive metastatic process.

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.

This study was designed to compare immunoexpression of somatostatin receptors (SR)1-5 and dopamine receptors 2 (DR2) in pituitary and non-pituitary ectopic ACTH-producing tumours. A total of 35 patients were enrolled in the study including 21 with Cushing's disease (CD) and 14 with ACTH-ectopic syndrome (ACTH-ES) associated with carcinoid tumours of lungs (n=11), kidney (n=1), thymus (n=1) and with small-cell lung carcinoma (n=1). All patients with corticotropinomas exhibited expression of SR 1-5 dominated by SR-1 (57%). 81% of these tumours expressed two and more SR subtypes. DR2 expression was documented in 52% of the tumours and co-expression of DR2 with different SR subtypes in 29%. Neoplasms associated with ACTH-ES expressed SR with the predominance of SR-3 (72%). Half of these tumours co-expressed two and more SR subtypes. Expression of DR2 was demonstrated in 50% of the neoplasms associated with ACTH-ES; 38% of them co-expressed SR and DF2.

The results of an examination, treatment, and postoperative follow-up of 520 patients with pheochromocytomas who were operated on at the Endocrinology Research Center, Russian Academy of Medical Sciences, in 1957 to 2002 have been analyzed. The patients’ mean age was 39.3±9.2 years; 50 (9.6%) patients aged less than 16 years. There were 213 (41.0%) males in the total group and 32 (62.0%) in the pediatric group. Bilateral adrenal lesion was detected in 68 (13.1%), including 16 (32%) of the 50 children aged less than 16 years. Extraadrenal tumors were ascertained in 36 (6.9%). The concomitance of pheochromocytoma with hereditary syndromes was observed in 36 (6.9%) cases. Two hundred and sixty patients were followed up for 4 to 25 years (mean 8.4+1.9). Recurrence ofpheochromocytoma was revealed in 49 (18.8%) patients; the genuine recurrence (a tumor at the site of primary surgery or metastasis) was found in 16 (6.15%) patients. The genuine recurrence of pheochromocytoma was seen in 6 (12.0%) of the 50 children; its main cause was organ-preserving surgery for multicenter adrenal lesion.

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 devoted to the Type 2 multiple endocrine neoplasia syndrome

The article is devoted to the descripition of the rare case with Icenko-Cushing syndrome caused by multiple bilateral benign and malignant adrenocortical tumors.

Primary hyperparathyroidism (PHPT) is a disease of the endocrine system caused by excessive secretion of parathyroid hormone (PTH) and is characterized by a pronounced violation of calcium and phosphorus metabolism. More recently, in Russia, PHPT was detected extremely rarely and often hid under the mask of recurrent urolithiasis, generalized osteoporosis and other pathologies. As a result, patients did not receive adequate treatment, which in a certain percentage of cases led to the development of serious complications.The clinical cases of severe PHPT in postmenopausal women are presented in this article.Первичный гиперпаратиреоз (ПГПТ) — заболевание эндокринной системы, обусловленное избыточной секрецией паратиреоидного гормона (ПТГ) и характеризующееся выраженным нарушением обмена кальция и фосфора. Еще совсем недавно в России ПГПТ выявлялся крайне редко и часто скрывался под маской рецидивирующей мочекаменной болезни, генерализованного остеопороза и другой патологии. В результате пациенты не получали адекватного лечения, что в определенном проценте случаев приводило к развитию тяжелых осложнений. Приводим описание клинических случаев тяжелого ПГПТ у женщин в постменопаузе.

One of the most difficult in diagnostic and treatment options for endogenous Cushing is the ectopic ACTH syndrome, which causes the development of tumors of different histogenesis localization producing adrenocorticotropic hormone (ACTH), and much less - corticotropin hormone (CRH). ACTH-secreting tumors varied in location, morphological structure and the degree of malignancy. Most of these tumors are characterized by an aggressive course with a propensity to metastasize and relapse. The article presents data of the prevalence, pathogenesis of ectopic ACTH tumors localized in the thymus, analyzis of clinical, morphological features, the methods of diagnosis and treatment. Based on the current literature, the world and our own experience on the diagnosis and treatment of patients with ectopic ACTH syndrome with localization of hormone production in the thymus, we want to highlight the current state of the problem in order to create the most efficient algorithm for diagnostic search and treatment of this difficult group of patients.

Today subclinical Cushing's syndrome is the most widespread variant of hormonal activity of incidentaloma's hormonal activity. This pathology is defined as a mild intensity autonomous cortisol hypersecretion, not causing specific clinical signs of hypercorticism, but detectable biochemically as derangements of the hypothalamic-pituitary-adrenal axis function. Some clinical symptomatology of metabolic syndrome, such as obesity, impaired carbohydrate metabolism and hypertension, are peculiar to subclinical hypercorticism more than to population. As a result all these symptomatology could lead to increasing cardiovascular risk. Till now there isn't a definite opinion about the need of surgical treatment of present pathology. But there is evidence, that after removing of incidentalomas clinical symptomatology of metabolic syndrome are improving. For understanding all possible risks, connected with subclinical hypercorticism, it's necessary to study the origins of their arising and present about the most adequate screening tests.

Laparoscopic adrenalectomy is an excellent alternative to open surgery, while there are doubts in regard to laparoscopic treatment of pheochromocytoma due to its unsteady intraoperative hemodynamics. The goal of the study was to define optimal surgical approach to pheochromocytoma.A total of 99 patients with pheochromocytoma were operated from 1990 to 2002. Nine, 28, 40, and 22 patients were operated respectively through laparoscopic (group 1), thoracophrenotomic (group 2), lumbotomic (group 3), and laparotomic (group 4) approaches. Intraoperative parameters including those related with hemodynamic stability were studied. Postoperative analgesic medication, complications, and hospital stay were registered.Mean operative time was 132 +/- 49 min, 104 +/- 29 min, 81 +/- 30 min, and 129 +/- 40 min, respectively, in groups 1, 2, 3, and 4. Thoracophrenotomic approach had a minimal time from starting of adrenal dissection to central adrenal vein crossing on the right side and lumbotomic approach on the left side. Laparoscopy showed longest length of that period on both sides. However, we observed more stable intraoperative dynamics during laparoscopic adrenalectomy in comparison with any traditional open approaches on both sides. Mean blood loss was 178 +/- 112 ml, 410 +/- 255 ml, 314 +/- 163 ml, and 420 +/- 398 ml, respectively, in groups 1, 2, 3, and 4. Blood transfusions were required in 0%, 35.7%, 20%, and 13.6% of cases, respectively, in groups 1, 2, 3, and 4. Using laparoscopic approach resulted in significant decrease of prescription of opoids, postoperative hospital stay, and rate of postoperative complications. Among traditional approaches lumbotomy presented better postoperative results due to its less invasive nature.Laparoscopy is a method of choice to pheochromocytoma in experienced hands. Open approaches are still feasible. Among traditional approaches lumbotomy should be preferred. Thoracophrenotomy can be justified only for major tumors on the right side. Laparotomy is indicated in selected cases of extraadrenal pheochromocytoma.

At the present time topical diagnosis of ACTH-producing neuroendocrine tumors of different localization is the most challenging problem for endocrinologists around the world. Despite the significant improvement of existing diagnostic techniques, in 9-19% localization of the source of ectopic secretion ACTH re-mains undetected throughout life. Thus even regular using of routine laboratory tests and visual methods of diag- nosis do not allow to reveal the primary tumor. This article describes a case report, that is particular by a long period of ectopic ACTH syndrome detection. However, this demonstrates the importance of inten-sive and prolonged diagnosis of primary tumor, that can significantly reduce the morbidity and mortality of these patients and improve their prognosis.

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder involving a predisposition to tumor development. The common features of Beckwith-Wiedemann syndrome include omphalocele, macroglos- sia and macrosomia. The increased risk for neoplasia is concentrated in the first eight years of life. However, this case presents a late onset of adrenocortical cancer assosiated with Beckwith-Wiedemann syndrome.

The main difficulty of primary hyperparathyroidism in the surgical aspect, it is possible atypical location of the parathyroid glands and multiple nature of the lesion. Proposed significant number of methods for pre- operative and intraoperative for topical diagnosis in primary hyperparathyroidism and the search for new methods and continues to the present. The proposed method of topical diagnostic studies of PTH-FNAB has a high statistical parameters of sensi- tivity, specificity and predictive value. This method is needed as an additional parameter specifying, in the topical diagnosis of primary hyperparathyroidism, especially in the case of ambiguous and questionable data received basic methods of topical studies in primary hyperparathyroidism.

Ectopic hormonesecreting of cecum are rare. We report the case of a 52yearold woman with a typical clin ical picture of Cushing disease. Diagnostic tests confirmed ACTH dependent Cushing’s syndrome, which is caused by neuroendocrine tumor of the cecum. Due to the rarity of the disease, we believe it appropriate to share our experience of this group of patients.

Ectopic hormone-secreting pheochromocytomas are rare. Only case reports exist in the literature. Despite the large number of guides on diagnosis and treatment of pheochromocytoma, and Cushing syndrome, the extreme rarity of ectopic ACTH-syndrome caused by pheochromocytoma, and complexity of clinical cause numerous diagnostic errors leading to treatment failure. Therefore, we belive it appropriate to share our experience of this group of patients.

Topical diagnostics of parathyroids an important stage in preoperative inspection of patients with a primary hyperparathyreosis. Methods of diagnostics existing for today not always demand use of all methods for each patient. A research problem was studying of diagnostic value of each of research methods separately and their combinations. The obtained data testifies to high sensitivity and specificity of each of methods of preoperative topical diagnostics of a pathology of parathyroids at patients with a primary hyperparathyreosis. Sensitivity of US, the Scintigraphy, US + Scintigraphy and the Multispiral computer tomography (MSCT) has compounded – 96,8 %, 91,8 %, 99,2 % and 95,2 % accordingly. Specificity of US, the Scintigraphy, US+ Scintigraphy and a MSCT has compounded – 91,9 %, 84,2 %, 94,1 % and 94,1 % accordingly. Findings of investigation have taped absence of statistically significant differences in sensitivity and specificity parametres between US and «the gold standard» diagnostics of a pathology of parathyroids that allows to recommend US, as a unique method of primary topical diagnostics of a pathology of parathyroids at a primary hyperparathyreosis. However any doubts in diagnostic accuracy of US demand expansion of all complex of diagnostic actions scintigraphy, including an SPECT, and also MSCT, especially in case of atypical a locating of parathyroids. On the basis of findings of investigation the optimum diagnostic algorithm is developed at a primary hyperparathyreosis.

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

The article is devoted to recurrent pheochromocytoma in a female patient with type IIa multiple endocrine neoplasia

In recent years, there has been a significant increase in the number of patients with diabetes. According to WHO (1988), up to 5% of the world's population suffers from this disease. This is facilitated by stress, adynamia, excessive nutrition, uncontrolled drug therapy, viral infections, hereditary predisposition, adverse environmental conditions, developmental abnormalities, etc.

The article is devoted to the clinical recommendations of the Russian Association of Endocrinologists for the diagnosis and treatment of autoimmune thyroiditis in adults