D. G. Beltsevich

Endocrinology Research Center

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ГА

Г. А. Мельниченко

Endocrinology Research Center

ЕА

Е. А. Трошина

Ministry of Health of the Russian Federation

НС

Н С Кузнецов

Endocrinology Research Center

ВЭ

В Э Ванушко

Endocrinology Research Center

МЮ

М Ю Юкина

НМ

Н. М. Платонова

Endocrinology Research Center

M. Yu. Yukina

Ministry of Health of the Russian Federation

A. Chevais

Endocrinology Research Center

НГ

Н. Г. Мокрышева

Endocrinology Research Center

НВ

Н В Молашенко

Endocrinology Research Center

Cooperative Institutions

Endocrinology Research Center

156

Ministry of Health of the Russian Federation

134

Sechenov University

Faculdades Guarulhos

Medical Radiological Research Center

Russian Academy of Sciences

Russian Cancer Research Center NN Blokhin

P.A. Hertzen Moscow Oncology Research Institute

Pirogov Russian National Research Medical University

National Medical Research Center of Cardiology

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Draft of clinical guidelines for the diagnosis and treatment of primary hyperparathyroidism in adult patients

Endocrine Surgery (2023)

И. И. Дедов Г. А. Мельниченко Н. Г. Мокрышева Е. Н. Андреева М. Б. Анциферов

The article presents a draft of clinical recommendations for the diagnosis and treatment of primary hyperparathyroidism in adult patients, which provides a modern examination algorithm, discusses the basic principles of laboratory, instrumental diagnostics and treatment approaches.

10.14341/serg12790

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Citations (12)

Atypical and typical course of neurofibromatosis type 1 in combination with pheochromocytoma

Endocrine Surgery (2022)

M. Yu. Yukina Ekaterina Avsievich Antonina Pushkareva Nurana Nuralieva E. V. Bondarenko

Neurofibromatosis type 1 is a hereditary disease that has a multisystem character of organism damage, a wide variability of clinical manifestations, up to the almost complete absence of typical symptoms. Phenotypic manifestations, their expressiveness and heaviness can be varied even among members of the same family with identical mutations. One of the possible clinical manifestations of this pathology is pheochromocytoma, the development of which is associated with a high risk of developing life-threatening conditions. Timely diagnosis of the disease, the choice of treatment tactics for the patient, genetic testing of blood relatives can significantly improve the survival rate and prognosis of the disease. In this article, on the presented clinical examples of patients with a typical and atypical course of type 1 neurofibromatosis in combination with pheochromocytoma, the issues of managing patients with this pathology are outlined.

10.14341/serg12730

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Citations (2)

Surgical treatment of patients with secondary hyperparathyroidism in chronic renal failure

Problems of Endocrinology (2003)

В. Н. Сморщок Н С Кузнецов А М Артемова Liudmila Rozhinskaya D. G. Beltsevich

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.

Autotransplantation

Hypoparathyroidism

Parathyroid gland

10.14341/probl11761

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Molecular genetic markers and criteria for the prediction of adrenocortical carcinoma

Russian Journal of Archive of Pathology (2019)

Л С Селиванова Anna Roslyakova A. V. Bogolyubova А. С. Тертычный D. G. Beltsevich

Studies of the last decade have demonstrated that the morphological and immunophenotypic patterns of adrenocortical carcinoma (ACC) have a high heterogeneity in both the occurrence of various tumors and the development of a solitary tumor. Carcinogenesis of ACC, like most neoplastic processes, is associated with mutations in at least 15 driver genes, with a wide range of chromosomal aberrations, epigenomic changes, and alterations of the microRNA profile. According to the literature, isolated genetic damage is also insufficient for the manifestation of the malignant phenotype of adrenocortical cells. Knudson's two-hit hypothesis is implemented in at least germline mutations: the development of ACC requires a second genetic event occurring in somatic cells, which leads to inactivation of the second allele of the gene. ACC is an extremely heterogeneous disease, which determines the complexity of differential diagnosis with benign adrenocortical tumors and that of prediction of the clinical course. Another no less important issue is the lack of valid predictors for the efficacy of mitotane, the use of which may be associated with severe adverse effects.В исследованиях последнего 10-летия продемонстрирована высокая неоднородность морфологической и иммунофенотипической картины адренокортикального рака (АКР) как в случае возникновения различных опухолей, так и при развитии одного образования. Канцерогенез АКР, как и большинства неопластических процессов, связан с мутациями как минимум в 15 драйверных генах, широким спектром хромосомных аберраций, эпигеномных изменений, а также с изменениями профиля микроРНК. По данным литературы, для манифестации злокачественного фенотипа адренокортикальных клеток недостаточно изолированной генетической поломки. В случае как минимум герминальных мутаций реализуется теория двойного удара А. Кнудсона: для развития АКР требуется второе генетическое событие, произошедшее в соматических клетках, которое приводит к инактивации второго аллеля гена. АКР является чрезвычайно гетерогенным заболеванием, что определяет сложность дифференциальной диагностики с доброкачественными адренокортикальными опухолями и прогнозирования клинического течения. Не менее значимым вопросом является отсутствие надежных предикторов эффективности митотана, применение которого может быть ассоциировано с тяжелыми побочными эффектами.

Epigenomics

Mitotane

10.17116/patol20198105192

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Citations (5)

РЕЗУЛЬТАТЫ ИММУНОГИСТОХИМИЧЕСКОГО ИССЛЕДОВАНИЯ АНТИТЕЛ К CYP11B2 ПРИ ПЕРВИЧНОМ ГИПЕРАЛЬДОСТЕРОНИЗМЕ

Ожирение и метаболизм (2021)

Н Ю Романова Л С Селиванова Н. М. Платонова D. G. Beltsevich Е. А. Трошина

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Adrenokortikal'nyy rak

Endocrine Surgery (2009)

D. G. Beltsevich N S Kuznetsov V E Vanushko

Адренокортикальный рак (АКР) редкое заболевание, до настоящего момента характеризующееся поздним сроком выявления и неблагоприятным лечебным прогнозом.

10.14341/2306-3513-2009-1-14-16

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The clinical practice guidelines for primary hyperparathyroidism, short version

Problems of Endocrinology (2021)

Н. Г. Мокрышева Anna Eremkina Svetlana Mirnaya Julia Krupinova Iya А. Voronkova

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.

Endocrine disease

10.14341/probl12801

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Citations (46)

Management of patients with differentiated neuroendocrine tumors of jejunum and ileum (according to international guidelines)

Endocrine Surgery (2011)

D. G. Beltsevich Г. А. Мельниченко

Клинические рекомендации по ведению больных с нейроэндокринными опухолями дистальной части тонкой (тощей и подвздошной) кишки выработаны группой международных экспертов и освещают обновление эпидемиологических данных, пересмотр различных параметров классификации опухолей (ВОЗ, гистологические критерии, TNM), а также мультидисциплинарные проблемы, связанные с различными вопросами диагностики, стадирования и выбора оптимальных методов лечения больных с нейроэндокринными опухолями указанной локализации. Полный текст рекомендаций: B. Eriksson, G. Kloppel, E. Krennin, H. Ahlman, U. Plоckinger, B. Wiedenmann, R. Arnold, C. Auernhammer, M. Kоrner, G. Rindi, S. Wildi et al. Neuroendocrinology. 2008;87:8–19.

Jejunum

Neuroendocrine tumour

Neuroendocrinology

10.14341/2306-3513-2011-2-5-17

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Citations (2)

Forty-year experience in diagnosing and treating chromaffine tissue tumors

Problems of Endocrinology (2003)

И. И. Дедов Н. С. Кузнецов D. G. Beltsevich L. V. Kuratev

10.14341/probl11442

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Citations (2)