Problemy endokrinologii最新文献

Aim: To determine significant factors affecting the survival of patients with ectopic ACTH syndrome (EAS).

Materials and methods: A multi-center, observational study with a retrospective analysis of patients with EAS. The end point of the study was the fatal outcome of patients from various causes. In order to identify predictors of survival or mortality, univariate and multifactorial Cox regression analyses were carried out. ROC-analysis was used to determine the prognostic threshold values of individual predictors. The survival analysis was carried out using the Kaplan-Mayer method. Statistical data processing was carried out by using IBM SPSS Statistics 23.

Results: The age of patients at the time of diagnosis ranged from 12 to 76 years (Me 40 years [28;54]). The age of the studied population was 55 years [38; 64] for women and 42 years [32; 54] for men. The median period of observation was 50 months [13;91], with a maximum follow-up of 382 months. 92 patients (60,9%) had bronchopulmonary NET, 17 (11,3%) - thymic carcinoid, 8 - pancreatic NET, 5 -pheochromocytoma, 1- cecum NET, 1- appendix carcinoid tumor, 1 - medullary thyroid cancer and 26 (17,2%) patients had an occult NET. The primary tumor was removed in 101 patients (66,9%). Bilateral adrenalectomy was performed in 42 (27,8%) cases. Metastases were revealed in 23,2% (n=35) of patients. Relapse of the disease was observed in 24,4%, long-term remission was preserved in 64 patients (74,4%). Death occurred in 42 patients (28%). The average age of survivors was 47,0±15,2 versus 53,5±15,6 years for the deceased (p=0,022). The average survival time from diagnosis for the deceased was 32 months, Me 16,5 months [7;54]. Multivariate analysis revealed that the following factors have a direct impact on survival: age of diagnosis ≥51 years (OR 4,493; 95% CI 2,056-9,818, p<0,001), bronchopulmonary neuroendocrine tumor (NET) (OR 0,281; 95% CI 0,119-0,665, p=0,004), the presence of distant metastases (OR 2,489; 95% CI 1,141-5,427, p=0,022), late-night salivary cortisol (LNSC) ≥122,2 nmol/L (OR 2,493; 95% CI 1,014-6,128, p=0,047).

Conclusion: The prognosis of patients with EAS is influenced by the age of diagnosis, NET localization, distant metastases and level of LNSC. The most common cause of ectopic ACTH syndrome was bronchopulmonary NET which was associated with the best survival rate.

Today most adolescents have their first sexual experience at the age of 15-19. However, only 44% of girls and young women (15-24 years old) report about contraception at that moment. A decision on pregnancy in adolescence is a difficult choice and any scenario may cause serious medical and social problems. Complications after an artificial abortion have a negative impact on a woman's fertility. Diabetes mellitus type 1 and arterial hypertension accompanied with obesity within the metabolic syndrome are defined by the World Health Organization (WHO) as diseases, which increase risk of an unplanned pregnancy. The article consoders problems of interaction of a doctor and a teenage girl with endocrinopathy, when discussing her sexual health, the analysis of the literature reflecting the influence of contraception on the course of the underlying pathology is presented. The authors formed a list of drugs acceptable for use in diabetes and obesity based on assessment of risks and preferences from the use of different methods of fertility control. The work contains information about the procedure of starting contraception, the rules of future dynamic monitoring of the patient.

Background: Precise localization of abnormal parathyroid glands is important for a successful surgery for primary hyperparathyroidism (PHPT). While a large number of patients can be successfully treated with the focused parathyroidectomy, there is a considerable rate of the persistent PHPT mostly because of undetected multiglandular disease (MGD).

Aim: The aim of the study was to evaluate the meaning of preoperative visualization data for planning the surgery for patients with PHPT.

Materials and methods: The study was conducted at SPBU Hospital in 2017-2018. 810 patients who underwent a primary surgery for PHPT were included in the study. Preoperative imaging results were investigated and multivariative logistic regressions were calculated to assess the predictive values of preoperative data. The rate of cases with persistent disease and cases with MGD were compared between patients with different results of preoperative data.

Results: Age, sex, body mass index, negative results of preoperative US, MIBI and 4D CT were not independently associated with the higher risk of multiglandular disease. The larger number of performed preoperative visualization studies were associated with the higher risk of persistence. 37% cases of MGD were not identified preoperatively. There were 7 cases with previously unsuspected second adenomas found only due to bilateral neck exploration.

Conclusion: Any combination of preoperative visualization modalities was not able to rule out the MGD reliably. Efficacy of surgical treatment was not associated with the higher number of preoperative studies. Bilateral neck exploration may decrease the rate of the persistent hyperparathyroidism improving the identification of multiglandular disease.

Background: Differential diagnosis between the normocalcemic primary hyperparathyroidism (nPHPT) and secondary hyperparathyroidism (SHPT) due to hypercalciuria remains a challenge.

Aim: The aim of this study was to investigate the capability of short test with hydrochlorothiazide for the differential diagnosis of nPHPT and SHPT.

Materials and methods: A retrospective study was conducted with the participation of 28 patients who underwent a functional test with thiazide diuretics during hospitalization in the Department of parathyroid glands pathology and mineral disorders of the Endocrinology Research Centre, Russia. Parameters of mineral metabolism were evaluated before and 3-5 days after taking hydrochlorothiazide 50 mg/day.

Results: According to baseline and dynamic biochemical evaluation patients were divided into 3 groups. Group 1 (n=21) included patients with confirmed PHPT, who reached hypercalcemia accompanying with an elevated level of iPTH (n=19) or an increased level of iPTH accompanying with normocalcemia (n=2). In group 1, baseline Caadj. was 2.48 mmol/l [2.47; 2.52], iPTH 107.5 pg/ml [86.8; 133.0], after short test - 2.63 mmol/l [2.59; 2.66] and 102.1 pg/ml [95,7; 124,1]. Group 2 included only one who was diagnosed with SHPT, a normal value of iPTH with concomitant normocalcemia was achieved after 4 days of hydrochlorothiazide therapy (baseline Caadj. 2.35 mmol/l, iPTH 74.5 pg/ml vs at 2.27 mmol/l and 50.7 pg/ml respectively). Patients with doubtful results of the test entered in group 3 (n=6), they did not achieve significant changes in the calcium and iPTH levels, so it was recommended to continue the test on an outpatient basis (baseline Caadj. 2.39 mmol/l [2.33;2.45], iPTH 97.0 pg/ml [83.1;117.0]); after short test - 2.47 mmol/l [2.42; 2.48] and 91.3 pg/ml [86.9; 124.0] respectively). Groups with PHPT and SHPT and doubtful results significantly differed from each other in Caadj (р=0.003, U-test, Bonferroni correction Р0=0.006), but not in iPTH, daily calciuria, eGFR, and phosphorus. There were no significant differences in the incidence of classical complications of PHPT.

Conclusion: The diagnosis of PHPT was confirmed in 21/28 patients 3-5 days after taking hydrochlorothiazide 50 mg/day. The obtained results are significant for the differential diagnosis in hospitalized patients with an unspecified genesis of hyperparathyroidism.

Background: The issues of monitoring the effectiveness of iodine deficiency prevention programs are an important component in the process of iodine elimination. Neonatal thyrotropin (TSH) has been used as a criterion for the severity of iodine deficiency since 1994, however, the question of the "cut-off point" of the neonatal TSH level has been widely discussed in the recent literature.

Aim: Evaluate the criterion for neonatal hyperthyroidism above 5 mIU/l from the perspective of monitoring iodine deficiency and establish a «cut-off point» on the model of healthy pregnant women with adequate iodine status.

Materials and methods: A prospective study was conducted in a population of pregnant women in the city of Tyumen, with the formation of observation groups according to the level of iodine excretion in the urine - the main group (with adequate iodine status throughout the entire gestation period) and the comparison group (women with iodine levels less than 150 μg/l). The results of neonatal screening for congenital hypothyroidism in children of women participating in the study were evaluated. The frequency of neonatal TSH above 5mIU/l was assessed in the observation groups. ROC-analysis was performed and a «cut-off point» of the level of neonatal TSH was established as an indicator of iodine deficiency.

Results: The median urinary iodine concentration in the population of pregnant women in Tyumen was 159.05 μg/l, the incidence of goiter was 0.38%, the incidence of neonatal hyperthyroidism above 5 mIU/l was 2.88%, which characterizes adequate iodine intake in the pregnant population women. The frequency of neonatal TSH above 5 mIU/l in newborns from women from the main group was 1.47%, and in the comparison group - 9.3% (p = 0.076). ROC analysis revealed a threshold value of neonatal TSH of 2.77 mIU/l at the cut-off point, which corresponded to the highest value of the Youden index. Urinary iodine concentrations greater than 150 μg/l were predicted for nTSH values below this value.

Conclusion: Analysis of databases of neonatal screening for congenital hypothyroidism makes it possible to effectively, quickly and at minimal cost annually assess the iodine status in the population. The established «cut-off point» of neonatal TSH in the model of healthy pregnant women with adequate iodine intake in our work is 2.77 mIU/l, the absence of statistically significant differences in the incidence of neonatal hyperthyroidism above 5 mIU/l from women with different iodine status during pregnancy indicate the need to revise the existing threshold of 5 mIU/l and may be an incentive to conduct large-scale studies in regions with different iodine supply.

Often transfusions red blood cells in patients with hereditary anemias lead to iron overload, that can cause endocrine complications, such as growth retardation, hypothyroidism, hypogonadism, and disorders of carbohydrate metabolism.Clinical case 1. A boy with transfusion-dependent (TD) Diamond-Blackfan anemia at 16.3 years presented with impaired fasting glucose, growth hormone (GH) deficiency, hypogonadotropic hypogonadism; GH therapy was initiated. At the age of 16.8 years old secondary hypothyroidism, secondary hypocorticism and diabetes mellitus were diagnosed. At 17.2 years continuous glucose monitoring (CGM) detected glucose elevations up to 11.7 mmol/l. Therapy with GH and testosterone ethers was continued; levothyroxine and cortef were stopped by patient. At 17.9 years height was 163 cm; no data supporting hypothyroidism nor hypocorticism; glycaemia within goal range.Clinical case 2. A girl with TD beta-thalassemia major at the age of 11.5 years presented with GH deficiency; GH therapy has been conducted from 12.8 to 15.3 years of age. At 13.8 years retardation of pubertal development was diagnosed. At 15.0 hyperglycemia 7.2 mmol/l was detected; normal results of oral glucose tolerance test (OGTT) were observed; glycemia elevations were up to 9.5 mmol/l according to CGM data. At 16.0 height was 152 cm; because of pubertal development arrest hormone replacement therapy was prescribed.CONCLUSION. Growth, pubertal and carbohydrate metabolism disorders were diagnosed in patients with TD hereditary anemias, that confirms the necessity of regularly endocrine investigation. To detect impairment of carbohydrate metabolism investigation of fasting blood glucose, OGTT, and CGM is recommended; glycated hemoglobin measurement is not considered reasonable.

The article presents current data on the prevalence of thyroid diseases associated with iodine deficiency in Russia, focuses on the features of the comorbidity of iodine deficiency and autoimmune thyroid pathologies, methods for assessing the iodine supply of the population. Information about the study and prevention of iodine deficiency diseases (IDD) in the USSR and the Russian Federation is given. The history of legislative initiatives aimed at eliminating dietary iodine deficiency and preventing IDD is illustrated in detail. The ways of solving the problem of iodine deficiency at the present stage, both at the federal and regional levels, are proposed.

Turner syndrome (TS) is a chromosomal disorder affecting female and characterized by complete or partial monosomy of the X chromosome. These genetic changes lead to the abnormalities in growth and development and increase the risk of autoimmune diseases, including those affecting the thyroid. Thyroid pathology in TS may include autoimmune thyroiditis, hypothyroidism, thyrotoxicosis (Graves disease, AIT in the hyperthyroid state).Thyrotoxicosis is the clinical syndrome of excess circulating thyroid hormones. One of the main causes of thyrotoxicosis is Graves' disease (GD), an organ-specific autoimmune disease caused by the production of stimulating thyrotropin receptor antibodies. There are three treatment options for thyrotoxicosis: anti-thyroid drugs, radioactive iodine and thyroidectomy. A personalized approach to disease management is especially important in cases of genetic diseases.We present a clinical case of a patient with TS and GD, who has been referred to a radiologist at the Department of Radionuclide Therapy of Endocrinology Research Center. The patient was diagnosed with congenital hypothyroidism at neonatal screening, but thyroid hormones therapy was initiated aged three. Based on the survey, GD was diagnosed aged twenty one. Anti-thyroid drug therapy was started, which resulted in toxic hepatitis. Taking into account intolerance to anti-thyroid drugs, radioiodine therapy has been recommended, which led to hypothyroidism.

Background: Myokines are synthesized by myocytes and released into the bloodstream in response to muscle fiber contraction. They have a positive effect on carbohydrate and lipid metabolism, muscle mass growth, osteogenesis, increase tissue sensitivity to insulin, counteract inflammation of adipose tissue. The study of their secretion in response to physical activity (FA) can help to personalize the therapy of obesity.

Aim: to study the features of the secretion of myokines in children with constitutionally exogenous obesity during physical activity of different duration and intensity and to evaluate their relationship with the parameters of the body composition.

Materials and methods: 26 children (10 boys and 16 girls) were included in the study 15 [13; 16] years old, SDS BMI: +2.91 [2.24; 3.29], with sexual development according to Tanner 4-5. Two groups of 13 people were formed by random distribution. Group I performed FA (walking on a treadmill under the control of heart rate) of different duration: 30 and 60 minutes at the same intensity (less than 3 metabolic equivalents (MET)). Group II - FA of different intensity: low - less than 3 METH and moderate - 3-6 METH with the same duration of 45 minutes. Commercial kits for enzyme immunoassay were used to determine the level of myokines. The assessment of the compositional composition of the body was carried out by bioimpedance analysis (analyzer In Body 770, South Korea) in the morning, on an empty stomach. Statistical processing was carried out using STATISTICA v.12.0 (StatSoftInc., USA). The results are presented in the form of median (Me) and quartiles (Q1; Q3) corresponding to 25 and 75 percentiles. The critical significance level (p) was assumed to be <0.05.

Results: moderate intensity FA leads to a maximum increase in the level of myokines: interleukin-6 (IL-6) by 215.7% and decorin by 34.3%, a decrease in the level of irisin by 16.5%. An hour-long low-intensity workout leads to a moderate increase in the level of IL-6 by 80.5%, to a decrease in the level of irisin by 31.1%. Myostatin increases equally both after 60-minute FA and after moderate intensity FA by 30.9% and 31.8%, respectively. Short low-intensity FA (lasting 30 minutes) it is not accompanied by a significant increase in the expression of myokines. The relationship between the amount of muscle (r=0.65), lean (r=0.62), fat-free mass (r=0.64) and the level of decorin after FA was noted. There was no statistically significant relationship between the parameters of the body composition and the levels of IL-6, myostatin, and irisin. There were no gender differences in both basal and stimulated myokine secretion.

Conclusion: Moderate intensity FA and low intensity 60-minute FA are most effective for obese children. A 30-minute low-intensity FN is insufficient to increase the secretion of myokines by skeletal muscles.

A severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has rapidly spread around the world since was first scientifically described in December 2019. At present approximately 400 million people have suffered from the disease, almost 6 million people have died.SARS-CoV-2 uses the angiotensin-converting enzyme 2 (ACE2) and the serine protease TMPRSS2 for S protein priming. ACE2 and TMPRSS2 are expressed in several endocrine glands, including the pituitary, pancreas, thyroid, ovaries, and testes. Thus, the endocrine glands may be a direct target for SARS-CoV-2. The main risk factors for severity of the COVID-19 are obesity, arterial hypertension, diabetes mellitus (DM), vertebral fractures, which potentially predisposes patients to a severe course of COVID-19.In this review, we present current data on the course of COVID-19 in patients with hypothalamic-pituitary diseases, and also discuss treatment for endocrinopathies during to COVID-19.