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Vol 66, No 3 (2020)

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Editorial notice
Organization, problems and development prospects of endocrinology care and specialist training
Pigarova E.A.

Currently, among the priority tasks of the health care system is to improve the quality of medical care provided to the population and the accessibility of services. Incomplete staffing is a serious slowdown to the provision of quality care. The shortage of medical personnel is widespread in medicine, which is especially pronounced in primary health care, and endocrinology is no exception. The shortage of personnel is directly related to the problems of accessibility and quality of medical care. Reorganizing the system of professional training, retraining and continuing medical education in a volume that allows eliminating quantitative and qualitative defects in providing care for people with endocrine diseases, as well as analyzing staffing needs and planning based on the real needs of the population, will help to achieve the solution to the set tasks of the healthcare system.

Problems of Endocrinology. 2020;66(3):4-5
Clinical endocrinology
Use of thyroid hormones in the treatment of cardiovascular diseases: literature review
Borisov D.V., Gubaeva D.N., Praskurnichiy E.A.

Cardiovascular diseases remain the leading cause of death all over the world. Thyroid hormones play a significant role in the regulation of cardiac function. According to a number of researches, patients with cardiovascular diseases usually have a decrease in the concentration of thyroid hormones in the blood serum, which may be associated with a poor prognosis. Today it still remains unclear whether the change in the bioavailability of thyroid hormones in the myocardium is a favorable physiological mechanism or a replication of an adaptation disorder. Experimental researches suggest that thyroid hormone therapy may be applied in clinical cardiology.

This review describes the results of researches examining the use of thyroid hormones in patients with cardiovascular diseases, as well as experiment data on animal models. The available data on the use of thyroid hormones in patients with acute myocardial infarction and heart failure allow us to suggest that normalization of thyroid hormone levels is a safe and potentially effective treatment method in the group of patients with cardiovascular disease. At the same time, the data on the use of thyroid hormones in patients who have undergone an open-heart surgery or heart transplantation are limited. However, at present, it is difficult to draw unambiguous conclusions about the benefits, as well as about the possible risk of using thyroid hormones in the described conditions. Large-scale clinical researches are required to confirm the safety and evaluate the effectiveness of such therapy. Moreover, it is necessary to set parameters for evaluating the safety and effectiveness and understand which hormone (thyroxine or triiodothyronine), what dosage and at what stage of the disease should be applied. Until we do not have answers for these questions, thyroid hormone therapy in patients with cardiovascular diseases should remain within the research field.

Problems of Endocrinology. 2020;66(3):6-14
Changes in the morphological structure of giant prolactinoma during treatment with cabergoline
Astafyeva L.I., Shishkina L.V., Kadashev B.A., Kalinin P.L., Sidneva J.G., Sharipov O.I., Melnichenko G.A.

Currently, cabergoline therapy is the main method of treatment with prolactin. The use of the drug in most cases leads to tumor regression, normalization of prolactin levels and restoration of gonadotropic function. The mechanism of its impact on tumor cells in vivo, which is dynamically traced in the same human tumor, is the case of considerable interest. We observed a 30-year-old patient who was operated on twice for a giant prolactinoma before and on treatment by cabergoline. The morphological study after the first surgery (before introducing of cabergoline therapy) revealed a prolactin-positive pituitary tumor with a Ki-67 labeling index of 8% and with strong expression of dopamine type 2 receptors (D2R), CD31 and CD34. After 4 months, during which the patient received cabergoline at a dose starting from 0.5 mg to 1.5 mg per week, a second transsphenoidal surgery was performed with subtotal removal of residual tumor tissue. During the morphological study of the second biopsy sample, the tumor retained a pronounced immunopositivity to prolactin and D2R, with a decrease in the labeling index Ki-67 to 2%, as well as a decrease in the expression of CD31 and CD34. Subsequent cabergoline therapyresulted in persistent normoprolactinemia, restoration of androgen (and reproductive) status, and no tumor recurrence over a 10-year period on cabergoline treatment. Thus, one of the mechanisms of effect of cabergoline that leads to tumor regression is a decrease in the proliferative index and angiogenesis of the tumor.

Problems of Endocrinology. 2020;66(3):15-21
Graves’ disease in HIV patient receiving antiretroviral therapy
Morgunova T.B., Zorina A.A., Maloletkina E.S., Sytch Y.P., Vasileva A.V., Fadeyev V.V.

The article focuses on the clinical case of Graves’ disease in a patient with HIV infection who is receiving antiretroviral therapy. The number of HIV-infected patients has increased significantly in recent decades all over the world. The currently used highly active antiretroviral therapy can significantly improve the prognosis for these patients. However, its use is associated with a number of complications, in particular the development of immune reconstitution syndrome, under which the development of such autoimmune diseases as Graves’ disease, polymyositis and Guillain-Barre syndrome may occur. Therefore, we would like to draw the attention of doctors to the possibility of such a complication in patients receiving antiretroviral therapy. Timely diagnosis and treatment of thyroid disorders will help to avoid the complications associated with an excess or deficit of thyroid hormones.

Problems of Endocrinology. 2020;66(3):22-26
Radioiodine therapy outcome in toxic multinodular goiter patient with concomitant hereditary Hasharon hemoglobinopathy
Dzeytova D.S., Shklyaev S.S., Rumyantsev P.O., Sheremeta M.S., Trukhin A.A., Tsvetaeva N.V., Kozhedub E.E.

This research describes a clinical case of treatment of a patient with thyrotoxicosis with concomitant hematological pathology – carriage of unstable hemoglobin Hasharon. A patient diagnosed with «Diffuse toxic nodular goiter. Thyrotoxicosis of medium severity. Drug-induced hypothyroidism» was admitted to the Department of radionuclide therapy for the purpose of treatment with radioactive iodine. Onset of disease - summer 2018 (thyroid-stimulating hormone (TSH) – 0 mIU/ml). The instrumental studies (ultrasound, scintillation scanning of the thyroid gland) were performed at the pre-radioiodine therapy (RIT) diagnostic stage. The history of the disease indicates, that in 2000 the patient was suspected of having abnormal hemoglobin, since then no examinations have been conducted and anemia has never been detected. The diagnosis of ancestral hemoglobinopathy with the presence (17%) of unstable Hasharon-Sinai-Sealy hemoglobin in a heterozygous form was verified during the preparation to RIT. The radionuclide therapy I131 with activity of 400 MBq was performed on 02.07.2019. The monthly monitoring of laboratory and instrumental indicants was carried out during the post-therapeutic period: the state of hypothyroidism was reached by the end of 2 months after RT, no episodes of significant increase in bilirubin levels were observed during the observation period; no side effects from RT were stated. It becomes possible based on the example of the above observation, to judge the safety of conducting RT for treatment of thyrotoxicosis in patients with similar hemoglobinopathy, without excluding, however, the need for an individual approach in each case.

Problems of Endocrinology. 2020;66(3):27-32
The role of molecular testing in thyroid tumors
Kachko V.A., Platonova N.M., Vanushko V.E., Shifman B.M.

¹I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia; ²Endocrinology Research Centre, Moscow, Russia

Thyroid cancer is the most common endocrine gland cancer. In the last few decades, the molecular diagnostics for thyroid tumors have been widely researched. It is one of the few cancers whose incidence has increased in recent years from microcarcinomas to common, large forms, in all age groups, from children to the elder people. Most researches focus on the genetic basis, since our current knowledge of the genetic background of various forms of thyroid cancer is far from being complete. Molecular and genetic research has several main directions: firstly, differential diagnosis of thyroid tumors, secondly, the prognostic value of detected mutations in thyroid cancer, and thirdly, targeted therapy for aggressive or radioactive iodine-resistant forms of thyroid cancer. In this review, we wanted to update our understanding and describe the prevailing advances in molecular genetics of thyroid cancer, focusing on the main genes associated with the pathology and their potential application in clinical practice.

Problems of Endocrinology. 2020;66(3):33-46
Cushing’s syndrome due to bilateral oncocytic adrenal tumor
Kim E.I., Mamedova E.O., Selivanova L.S., Buryakina S.A., Gorbunova N.P., Latkina N.V., Kuznetsov N.S., Belaya Z.E.

Cushing’s syndrome accounts for approximately 20–30% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushing’s syndrome due to bilateral adrenal tumors is extremely rare. Adrenal oncocytomas are another rare cause of endogenous hypercortisolism: about 13 cases are described in the literature. Oncocytomas are rare epithelial neoplasms, characterized by abnormally excessive accumulation of defective mitochondria in the cytoplasm of cells, and make up 1.8% of all adrenal neoplasms.

We describe a 58-year old patient with Cushing’s syndrome and bilateral adrenal tumors. Multispiral computed tomography of the adrenals showed signs suspicious of lipid-poor atypical adenomas or malignant tumors. Surgical treatment was the method of choice, and the larger tumor was excised first. Due to the absence of remission of endogenous hypercortisolism the excision of the second tumor was performed. Morphological and immunohistochemical examination confirmed the diagnosis of bilateral oncocytic adrenocortical tumors with uncertain malignant potential. Cases of bilateral hormone-producing adrenal oncocytomas have not been described in the literature.

Problems of Endocrinology. 2020;66(3):47-55
Carbohidrates metabolism disturbancies
Screening for gestational diabetes due to of the COVID-19 pandemic
Torosyan A.O., Loginova E.V., Gagaev C.G.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a virus that causes coronavirus disease in 2019 (COVID-19). Currently, there is no evidence that pregnant women are more vulnerable to COVID-19. All concerns and anticipated risks are related to the potential impact of COVID-19 on perinatal outcomes, so pregnant women require special attention in relation to the preventive measures, diagnosis and treatment of a new coronavirus disease. Women with gestational diabetes mellitus (GDM) belong to the group of high perinatal risk and need timely medical assistance. During the COVID-19 pandemic, there is a necessity in temporary changes of approaches to diagnosing GSD and pregnancy care before and after delivery in women with GSD. The purpose of our review is to present and analyze all available GSD screening recommendations, updated and published in various countries in response to the coronavirus pandemic, at the time of publication of this article. It seems that there is no single universal strategy to achieve a reasonable balance. In this regard, it is necessary to develop new national algorithms for GSD screening, taking into account both demographic factors and the features and capabilities of our health system. We believe that the knowledge and experience achieved as a result of these changes will lead to the revision and improvement of national and international recommendations.

Problems of Endocrinology. 2020;66(3):56-61
Pediatric Endocrinology
Clinical and molecular characteristics of patients with 46,XY DSD due to NR5A1 gene mutations
Kalinchenko N.Y., Kolodkina A.A., Raygorodskaya N.Y., Tiulpakov A.N.

Steroidogenic factor 1 (SF1, NR5A1) is a nuclear receptor that regulates multiple genes involved in adrenal and gonadal development, steroidogenesis, and the reproductive axis. Human mutations in SF1 were initially found in patients with severe gonadal dysgenesis and primary adrenal failure. However, more recent case reports have suggested that heterozygous mutations in SF1 may also be found in patients with 46,XY partial gonadal dysgenesis and underandrogenization but normal adrenal function. We have analyzed the gene encoding SF1 (NR5A1) in a cohort of 310 Russian patients with 46,XY disorders of sex development (DSD). Heterozygous SF1 variants were found in 36 out of 310 (11.6%) of cases, among them 15 were not previously described. We have not found any phenotype-genotype correlations and any clinical and laboratory markers that would allow to suspect this type of before conducting molecular genetic analysis.

Problems of Endocrinology. 2020;66(3):62-69
Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus
Shiryaev N.D., Kagantsov I.M., Sizonov V.V.

It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term «sexual maturity disorders.» Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

Problems of Endocrinology. 2020;66(3):70-80
Continuous subcutaneous infusion of somatostatin analogues in the treatment of congenital hyperinsulinism
Melikyan M.A., Gubaeva D.N., Kareva M.A.

BACKGROUND: Congenital hyperinsulinism (CHI) is a severe disease with a high risk of development of neurological complications due to persistent hypoglycemia. The use of an analog of somatostatin (octreotide) in patients with the resistance to the first-line drug allows to avoid surgical intervention. However, the octreotide is currently used in the form of frequent fractional injections due to the short duration of it’s effect. We present in this article our own experience of using octreotide in continuous subcutaneous infusion in pediatric patients in order to improve the quality of life.

AIM To evaluate the efficiency and safety of the regime of continuous subcutaneous infusion of octreotide with the use of micro-dispensers (pumps) in children with diazoxide-resistant course of CHI.

MATERIALS AND METHODS: An observational single-centre dynamic research was carried out on the basis of the Federal State Budgetary Institution «Endocrinology Research Centre» of the Ministry of Health of the Russian Federation. The study included pediatric patients with CHI and proven diazoxide-resistant course who were initially treated with octreotide in the form of intermittent subcutaneous injections. The researches compared the indicants of efficiency and safety of therapy on treatment of intermittent injections and after transfer to continuous subcutaneous infusion of the drug. The duration of each method of administration was at least 2 weeks.

RESULTS: 16 patients took part in the research. The median for the total duration of octreotide usage in the examined patients was 3 months. According to the results of the work, the use of micro-dispensers for continuous subcutaneous administration of octreotide allowed to reduce the number of patients with episodes of hypoglycemia for more than 4 times (13/16 vs. 3/16); p=0,001). Also, there was a significant decrease in the number of patients with hyperglycemic episodes (4/16 vs. 0/16); p=0.000) and reduced dose of intravenous glucose (6.8 vs 5.2 mg/kg/min; p=0.042) as a result of continuous therapy, which indicates the advantages of smooth continuous administration comparing to single injections. We have not detected any significant side effects of the treatment. Elevated liver enzyme levels, dyspeptic symptoms and gallstone formation in some patients did not require cancellation of therapy. There were no hormonal disorders in the form of hypothyroidism and somatotropic hormone deficiency against the background of continuous octreotide infusion.

CONCLUSIONS: Thus, the use of octreotide in patients with diazoxide-resistant course of СHI in continuous subcutaneous infusion using pumps has a number of advantages over the standard method of intermittent subcutaneous injection. This method of administration allows to achieve better glycemic control and reduce the risks from infusion therapy with highly concentrated glucose solutions, which undoubtedly improves the quality of life of patients.

Problems of Endocrinology. 2020;66(3):81-87

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