BACKGROUND: The majority of Kallmann patients have anosmia or hyposmia. This is how the disease is diagnosed. Some of them don’t have such complaints but olfactory dysfunction is diagnosed via olfactometry. Nowadays there is the lack of information about correlation between olfactometry results and subjective complaints. Correlation between olfactory bulbs size and olfactory dysfunction has been little studied. AIM: To explore olfactory bulb size and olfactory function in patients with congenital isolated hypogonadotropic hypogonadism. To correlate olfactory bulb sizes and smell test scores. MATERIALS AND METHODS : Single-centre comparative study. 34 patients were included. The main group consisted of 19 patients with hypogonadotropic (15 –with Kallmann syndrome, 4 — with normosmic hypogonadism). Olfactory bulbs MRI were provided to all the patients, olfactory test (Sniffin’ Sticks Test) and molecular-genetic studies were provided in all patients with hypogonadism. Control group consisted of 15 patients who were provided with orbits MRI. Olfactory bulbs were evaluated additionally in them. RESULTS: Normal size of olfactory bulbs were only in 1 patient with hypogonadism. Olfactory bulbs height and width were significantly smaller in patients with hypogonadism in comparison with control group (p<0.01). Height median of right bulb was 1.0 mm [0.2; 1.8] in patients from the main group vs. 3.0 [2.5; 3.2] in controls, width median of right bulb was 1.0 mm [0.2; 1.9] in patients from the main group vs. 2.5 [2.0; 3.0] in controls. Height median of left bulb was 0.8 mm [0.0; 1.2] in patients from the main group vs. 3.0 [2.7; 3.2] in controls, width median of left bulb was 0.8 mm [0.0; 1.2] in patients from the main group vs. 2.5 [2.0; 3.0] in controls. Correlation has been established between left bulb height (r=0.59) and width (r=0.67) and olfactometry results (p<0.05). 4 patients had no anosmia complaints but had olfactory dysfunction according to Sniffin’ Sticks Tests. CONCLUSION: Olfactometry was able to diagnose olfactory dysfunction in 78.5% (i.e. in 15 out of 19 patients with congenital isolated hypogonadotropic hypogonadism. However, anosmia complaints had only 11 out of 19 patients. It is the first results of olfactory bulb sizes in patients with hypogonadotropic hypogonadism in Russia. Uni — or bilateral hypoor aplasia were diagnosed in 94.7% patients with hypogonadism regardless of olfactory dysfunction. Bilateral olfactory bulbs hypoplasia were the most common MRI-finding (36.8%). Unilateral hypoor aplasia was diagnosed in 31.6% patients.

ОБОСНОВАНИЕ ОБОСНОВАНИЕ. В подавляющем большинстве пациенты с синдромом Кальмана отмечают неспособность различать запахи, что помогает своевременно установить диагноз. Некоторые из пациентов с синдромом Кальмана не предъявляют подобных жалоб, но при помощи ольфактометрии с использованием специальных наборов пахучих веществ у них выявляются нарушения обоняния. Нарушения обоняния часто отмечаются у пациентов с гипоплазией или аплазией одной или обеих обонятельных луковиц (ОЛ). Характер взаимосвязи размеров обонятельных луковиц и гипоили аносмии по результатам ольфактометрии у пациентов с ВИГГ в настоящее время мало изучен. ЦЕЛЬ ЦЕЛЬ. Изучить размеры ОЛ и обонятельную функцию у детей с ВИГГ. Установить наличие взаимосвязи между размерами ОЛ и обонятельной функцией. МАТЕРИАЛЫ И МЕТОДЫ МАТЕРИАЛЫ И МЕТОДЫ. Одноцентровое одномоментное сравнительное исследование. В исследование включены 34 пациента. Основную группу составили 19 детей с гипогонадотропным гипогонадизмом (15 — с синдромом Кальмана, 4 — с нормосмическим гипогонадотропным гипогонадизмом). Всем пациентам проводилась МРТ головного мозга с оценкой размеров ОЛ, ольфактометрический тест (Sniffin’ Sticks Test) и молекулярно-генетические исследования. Контрольную группу составили 15 детей, у которых при проведении МРТ орбит дополнительно оценивали размеры ОЛ. РЕЗУЛЬТАТЫ РЕЗУЛЬТАТЫ. Из 19 пациентов с ВИГГ нормальные размеры ОЛ имелись только у 1 пациента. У детей с гипогонадизмом высота и ширина ОЛ оказались достоверно меньше (p<0,01) в сравнении с контролем. Медиана высоты правой луковицы (ПЛ) у пациентов с ВИГГ составила 1,0 мм [0,2; 1,8] против 3,0 [2,5; 3,2] в контрольной группе, медиана ширины ПЛ — 1,0 мм [0,2; 1,9] против 2,5 [2,0; 3,0], медиана высоты левой луковицы (ЛЛ) у пациентов с ВИГГ — 0,8 мм [0,0; 1,2] против 3,0 [2,7; 3,2], медиана ширины ЛЛ — 0,8 мм [0,0; 1,2] против 2,5 [2,0; 3,0]. Выявлена корреляция между высотой (r=0,59) и шириной (r=0,67) левой ОЛ и результатами ольфактометрического теста (p<0,05). У 4 пациентов субъективная оценка обонятельной функции не совпала с данными ольфактометрического исследования. ЗАКЛЮЧЕНИЕ ЗАКЛЮЧЕНИЕ. Ольфактометрия позволила выявить нарушения обоняния у 78,5% пациентов с изолированным гипогонадотропным гипогонадизмом (15 из 19 пациентов), при этом субъективно на нарушения обоняния жаловались только 11 из 19 пациентов. Впервые в РФ представлены размеры ОЛ у пациентов с изолированным гипогонадотропным гипогонадизмом. У 94,7% пациентов вне зависимости от нарушения обонятельной функции отмечалась гипоплазия и/или аплазия одной или обеих ОЛ. Чаще всего встречалась гипоплазия обеих луковиц (36,8%), изменения ольфакторных луковиц (гипоплазия или аплазия) с одной стороны имелись у 31,6% пациентов.

ЦЕЛЬ: представление клинического опыта по диагностике, лечению и реабилитации пациентов с опухоль-индуцированной остеомаляцией. МАТЕРИАЛЫ И МЕТОДЫ: в наблюдение были включены 40 пациентов с клинически диагностированной опухоль-индуцированной остеомаляцией, у 34 из которых опухоль была локализована, 27 были прооперированы и 21 достигли стойкой ремиссии. РЕЗУЛЬТАТЫ: медиана возраста составила 48 [41 ; 63] на момент диагностики , 43% мужчины, время от первых симптомов до установления диагноза составило 8 [4 ; 10] лет. Лабораторно у пациентов отмечались гипофосфатемия 0,47 [0,4 ; 0,53] ммоль/л, снижение индекса реабсорбции фосфатов 62 [52 ; 67]%, и повышение щелочной фосфатазы 183 [112 ; 294] Ед/л. На момент установления диагноза 100% имели множественные патологические переломы, передвигаться полностью самостоятельно могли лишь 10%, при этом все испытывали болевой синдром, в том числе 77,5% охарактеризовали боль как нестерпимую (8-10 баллов по 10-балльной шкале). Среди методов, используемых для обнаружения опухолей, самыми чувствительными оказались сцинтиграфия с тектротидом с ОФЭКТ/КТ 71,4% (20/28) и МРТ 90% (18/20). В 35% случаях опухоль была локализована в мягких тканях и в 65% в костной ткани; при этом наиболее часто опухоль выявлялась в нижних конечностях, далее по частоте локализации была голова. Из 40 человек у 18 пациентов, в настоящее время, отсутствует ремиссия и пациенты получают консервативное лечение (препараты фосфора и альфакальцидол n=15 и бурозумаб n=3). В случае достижения ремиссии (n=21), наблюдался регресс клинической симптоматики и восстановление костной и мышечной масс. Широкое иссечение опухоли без предварительной биопсии приводило к наилучшему проценту ремиссии 87%. ВЫВОДЫ: опухоль-индуцированная остеомаляция наиболее часто встречается у лиц средней возрастной группы, характеризуется тяжёлым поражением костной и мышечной ткани с развитием множественных переломов, мышечной слабости и выраженного болевого синдрома. При лабораторной диагностике следует обращать внимание на гипофосфатемию, снижение индекса реабсорбции фосфатов и повышенную щелочную фосфатазу. Применение методов функциональной диагностики с меченным аналогом соматостатина к рецептору 2 подтипа и МРТ нижних конечностей и головы с контрастным усилением являются наиболее точными методами топической диагностики. В случае локализации опухоли рекомендуется широкое иссечение без предварительной биопсии.

Abstract Disclosure: S. Gronskaia: None. Z. Belaya: None. L. Rozhinskaya: None. T. Dubovitskaya: None. E. Mamedova: None. E.A. Pigarova: None. M. Degtyarev: None. S. Rodionova: None. Y. Buklemishev: None. D. Babaeva: None. V. Vladimirova: None. N. Tarbaeva: None. S. Serzhenko: None. A. Grigoriev: None. L. Dzeranova: None. V. Karpenko: None. A. Karasev: None. R. Fedotov: None. I. Ulyanova: None. D. Trukhina: None. N. Toroptsova: None. O. Lesnyak: None. N.G. Mokrysheva: None. G.A. Melnichenko: None. I. Dedov: None. Introduction: Tumor-induced osteomalacia is an acquired rare disease manifested by hypophosphatemic osteomalacia due to excessive secretion of fibroblast growth factor 23 (FGF23). FGF 23 is a non-classical hormone secreted by bone tissue (osteocytes) and regulates phosphorus metabolism.The aim of this work is to present clinical experience in the diagnosis, treatment and rehabilitation of patients with tumor-induced osteomalacia. Materials and methods: 40 patients with clinically-confirmed tumor-induced osteomalacia were included in the study, 34 of whom had the tumor localized, 27 underwent surgical treatment and 21 achieved stable remission. Results: The average age was 48 +/- 14 years, 43% were men, the time left from the the onset of the disease was 8 +/- 6 years. Biochemical fundings were hypophosphatemia 0.47 [0.4 ; 0.53] mmol/l, a decrease in the tubular reabsorption phosphate 62 [52 ; 67]%, and an increase in alkaline phosphatase of 183 [112 ; 294] units/l. At the time of diagnosis, 100% had multiple pathological fractures, only 10% could move independently, and 76.3% classified the pain as unbearable (8-10 points according to the 10-point pain syndrome scale ). Among the methods used to detect tumors, the most sensitive were scintigraphy with tectrotide with SPECT/CT 71.4% (20/28) and MRI 90% (18/20). In 55% of cases, the tumor was localized in soft tissues and in 45% in bone tissue; The tumor was most often detected in the lower extremities, followed by the head in frequency of localization. 18 patients currently have no remission and they receive conservative treatment (phosphorus and alfacalcidol n=15 and burozumab n=3). In case of achieving remission (n=21), regression of clinical symptoms and restoration of bone and muscle mass was observed. Extensive excision of the tumor without prior biopsy resulted in the best percentage of remission - 87%. Conclusion: Tumor-induced osteomalacia is most common in the middle age people. It is characterized by severe damage to bone and muscle tissue with the development of multiple fractures, muscle weakness and severe pain syndrome. In laboratory diagnostics, attention should be paid to hypophosphatemia, a decrease in the tubutar reabsorption phosphate index and increased alkaline phosphatase. The use of functional diagnostic methods with a labeled somatostatin analogue to the subtype 2 receptor and MRI with contrast enhancement are the most accurate methods of topical diagnostics. In case of localization of the tumor, a wide excision without a preliminary biopsy is recommended. Presentation: Thursday, June 15, 2023

Introduction: Tumor-induced osteomalacia is an acquired rare disease manifested by hypophosphatemic osteomalacia due to excessive secretion of fibroblast growth factor 23 (FGF23). FGF 23 is a non-classical hormone secreted by bone tissue (osteocytes) and regulates phosphorus metabolism. The aim of this work is to present clinical experience in the diagnosis, treatment and rehabilitation of patients with tumor-induced osteomalacia. Materials and methods : 40 patients with clinically-confirmed tumor-induced osteomalacia were included in the study, 34 of whom had the tumor localized, 27 underwent surgical treatment and 21 achieved stable remission. Results: The median age was 48 [41; 63] years, 43% were men, the time left from the the onset of the disease was 8 [4; 10] years. Biochemical findings were hypophosphatemia 0.47 [0.4; 0.53] mmol/l, a decrease in the tubular reabsorption phosphate 62 [52; 67]%, and an increase in alkaline phosphatase of 183 [112; 294] units/l. At the time of diagnosis, 100% had multiple pathological fractures, only 10% could move independently, and 77.5% classified the pain as unbearable (8–10 points according to the 10-point pain syndrome scale ). Among the methods used to detect tumors, the most sensitive were scintigraphy with tectrotide with SPECT/CT 71.4% (20/28) and MRI 90% (18/20). In 35% of cases, the tumor was localized in soft tissues and in 65% in bone tissue; The tumor was most often detected in the lower extremities, followed by the head in frequency of localization. 18 patients currently have no remission and they receive conservative treatment (phosphorus and alfacalcidol n=15 and burosumab n=3). In case of achieving remission (n=21), regression of clinical symptoms and restoration of bone and muscle mass was observed. Extensive excision of the tumor without prior biopsy resulted in the best percentage of remission — 87%. Conclusion: Tumor-induced osteomalacia is characterized by severe damage to bone and muscle tissue with the development of multiple fractures, muscle weakness and severe pain syndrome. In laboratory diagnostics, attention should be paid to hypophosphatemia, a decrease in the tubular reabsorption phosphate index and increased alkaline phosphatase. The use of functional diagnostic methods with a labeled somatostatin analogue to the subtype 2 receptor and MRI with contrast enhancement are the most accurate methods of topical diagnostics. In case of localization of the tumor, a wide excision without a preliminary biopsy is recommended.

ЦЕЛЬ: представление клинического опыта по диагностике, лечению и реабилитации пациентов с опухоль-индуцированной остеомаляцией. МАТЕРИАЛЫ И МЕТОДЫ: в наблюдение были включены 40 пациентов с клинически диагностированной опухоль-индуцированной остеомаляцией, у 34 из которых опухоль была локализована, 27 были прооперированы и 21 достигли стойкой ремиссии. РЕЗУЛЬТАТЫ: медиана возраста составила 48 [41 ; 63] на момент диагностики , 43% мужчины, время от первых симптомов до установления диагноза составило 8 [4 ; 10] лет. Лабораторно у пациентов отмечались гипофосфатемия 0,47 [0,4 ; 0,53] ммоль/л, снижение индекса реабсорбции фосфатов 62 [52 ; 67]%, и повышение щелочной фосфатазы 183 [112 ; 294] Ед/л. На момент установления диагноза 100% имели множественные патологические переломы, передвигаться полностью самостоятельно могли лишь 10%, при этом все испытывали болевой синдром, в том числе 77,5% охарактеризовали боль как нестерпимую (8-10 баллов по 10-балльной шкале). Среди методов, используемых для обнаружения опухолей, самыми чувствительными оказались сцинтиграфия с тектротидом с ОФЭКТ/КТ 71,4% (20/28) и МРТ 90% (18/20). В 35% случаях опухоль была локализована в мягких тканях и в 65% в костной ткани; при этом наиболее часто опухоль выявлялась в нижних конечностях, далее по частоте локализации была голова. Из 40 человек у 18 пациентов, в настоящее время, отсутствует ремиссия и пациенты получают консервативное лечение (препараты фосфора и альфакальцидол n=15 и бурозумаб n=3). В случае достижения ремиссии (n=21), наблюдался регресс клинической симптоматики и восстановление костной и мышечной масс. Широкое иссечение опухоли без предварительной биопсии приводило к наилучшему проценту ремиссии 87%. ВЫВОДЫ: опухоль-индуцированная остеомаляция наиболее часто встречается у лиц средней возрастной группы, характеризуется тяжёлым поражением костной и мышечной ткани с развитием множественных переломов, мышечной слабости и выраженного болевого синдрома. При лабораторной диагностике следует обращать внимание на гипофосфатемию, снижение индекса реабсорбции фосфатов и повышенную щелочную фосфатазу. Применение методов функциональной диагностики с меченным аналогом соматостатина к рецептору 2 подтипа и МРТ нижних конечностей и головы с контрастным усилением являются наиболее точными методами топической диагностики. В случае локализации опухоли рекомендуется широкое иссечение без предварительной биопсии.

BACKGROUND: Observation of changes in the volume and size of the pancreas has a long history, however, the results of studies are still not unambiguous, the specific causes of changes in pancreatic volume, as well as their consequences, are not clear. According to some data, the decrease of pancreas volume in life expectancy is 35–45% in the population of patients with a long history of type 1 diabetes, and about 20–25% during the first year of the disease. Interestingly, in T1D in 20–45% of cases, the development of exocrine pancreatic insufficiency is noted, one of the manifestations of which is pancreatic atrophy, leading to a decrease in life expectancy.AIM: Assess the volume and size of the pancreas, as well as factors that can influence on their changes.MATERIALS AND METHODS: The study included 78 patients with type 1 diabetes mellitus, the control group consisted of 23 people without previously identified disorders of carbohydrate metabolism, comparable in age and anthropometric parameters with the study group. RESULTS: The volume and dimensions of the pancreas were statistically significantly less in patients with T1D than in the control group. In addition, the influence of the duration of T1D and the age of onset of the disease on these indicators has been proven.CONCLUSION: The volume and size of the pancreas in patients with T1D is less than in healthy individuals. It is necessary to study the effect of these changes on the function of the pancreas.

McCune Albright syndrome (MAS) is a rare disorder caused by a sporadic postzygotic mutation in the GNAS gene, which encodes the alpha subunit of the Gs signaling protein.Permanent activation of the Gs protein leads to uncontrolled production of intracellular cAMP, and autonomic hyperfunction of target organs. Estimated prevalence of the disease – 1\100000 to 1\1000000. For the first time, MAS syndrome was described in 1936 as a triad of symptoms – fibrous dysplasia of bones (FD), spots on the skin of the color of «coffee with milk» and premature sexual development. However, it is now known, that the phenotype of the disease is much more complex. This is primarily due to the mosaic type of mutation in the GNAS gene, which determines a wide range of clinical manifestations, and presents certain difficulties in the diagnosis of this syndrome. Various endocrinopathies can be manifestations of MAS like an acromegaly, a hyperthyroidism, Cushing’s syndrome, hypophospatemic osteomalacia as well as damage to other organs and systems, namely the pathology of the gastrointestinal tract, hepato-biliary cardiovascular systems. This article presents three late diagnosed clinical cases of MAS in patients with coffee-milk spots and FD, multinodular goiter; in two cases in combination with acromegaly. We have demonstrated the possibilities of drug therapy for acromegaly in MAS, as well as the experience of using bisphosphonate for the treatment of FD.The knowledge of clinical manifestations of the MAS, early diagnostics and possibilities of drug therapy in the management of these patients have the important prognostic value for improving the quality of patient’s life.

Background. The level of urethrovaginal fusion (UVF) is an important criterion in choosing the tactics of surgical vaginoplasty in patients with urogenital sinus (UGS). The frequency of high UVR among all its forms is 5–10 % and considered the most difficult for surgical correction. There are few publications devoted to studies of the level of UVF in patients at puberty.Aim. To find a non-invasive method for diagnosing high UVF in patients at puberty with UGS.Materials and methods. In order to find a non-invasive method for diagnosing high UVF in this group of patients, we conducted a study based on a comparative assessment of endoscopic findings and data of magnetic resonance imaging (MRI). The study included 36 patients with UGS aged 12 to 34 years (average age 17 ± 5 years) who underwent surgical treatment at the National Medical Research Center of Endocrinology of the Ministry of Health of Russia from 2019 to 2022, including patients with congenital adrenal hyperplasia salt-wasting form (n = 24), viril form (n = 8), partial 46,XY gonadal dysgenesis (n = 2), mixed 45,X0/46,XY gonadal dysgenesis (n = 1), and partial androgen insensitivity syndrome (n = 1). All patients sequentially underwent MRI of the pelvis and endoscopy of the lower urinary tract with a time gap of 1 to 62 days.Results. We confirmed that high UVF in patients with UGS during puberty can be diagnosed in a non-invasive way, by pelvic MRI study. The length of the urethra of 22 mm or less corresponds to a high level of UVF, and the depth of UVF less than 32 mm practically excludes it.Conclusion. The method allows to clarify the severity of UGS in advance, before surgery, to determine the tactics of vaginoplasty and the risk of its complications, and to provide the patient and his relatives with more detailed information to obtain consent for the operation.

AIM:To present a clinical case of reversible hypopituitarism due to hypophysitis developed after COVID-19 infection.MATERIALS AND METHODS:A patient with residual clinical manifestations of hypopituitarism underwent clinical evaluation at the time of symptoms of hypopituitarism and in follow-up. Morning serum cortisol (171-536 nmol/l) was measured by electrochemiluminescence immunoassay. Morning ACTH (7.2-63.3 pg/ml), prolactin (66-436 mU/l), TSH (0.25-3.5 mIU/L), fT4 (9-19 pmol/l) and fT3 (2.6-5.7 pmol/l) were measured by chemiluminescence immunoassay. Data were analyzed throughout the course of the disease.RESULTS:A 35-year-old female developed clinical symptoms of hypopituitarism two months after recovery from a confirmed COVID-19 infection. Laboratory investigation confirmed hypocorticism, hypothyroidism, hypogonadism and the patient was prescribed appropriate hormonal therapy in January 2021. Four months later the symptoms were alleviated (April 2021) and there were signs of recovery shown by imaging and hormonal: morning serum cortisol 227 nmol/l, morning ACTH 33.96 pg/ml, prolactin 68.3 mU/l, TSH 2.626 mIU/L, fT4 10.75 pmol/l, fT3 3.96 pmol/l. Thyroid hormone was discontinued, but hypogonadism and hypocorticism persisted with estradiol - 51.48 pmol/l, 24h urine cortisol level - 41.8 nmol/day. MRI results showed that the signs of hypophysitis were alleviated in comparison with MRI from January 2021. Full recovery of pituitary axis was reported in October 2021, with recovery of normal menstrual cycle. Furthermore, hormonal profile was likewise normal.CONCLUSION:This report provides evidence of delayed damage to the pituitary gland after infection with the COVID-19, with recovery of its function and structure. To date, the mechanisms of such an impact are not entirely clear; further collection of data on such cases and analysis is required.

This article presents a literature review of the various forms of hypophysitis, its varieties, as well as the problem of radiation diagnosis and treatment of this pathology. Hypophysitis is a poorly understood and multifactorial disease which the difficulty of diagnosing is not only to a variety of nonspecific clinical manifestations and hormonal research data, but also the ambiguous results of MRI studies, the lack of clear MR patterns. The article reflects the main histological types of hypophysitis, the peculiarities of diagnosis in connection with general clinical symptoms, outlines the features of each type of hypophysitis with their own clinical observations. This review is devoted to modern ideas about the clinical course of hypophysitis, presented a set of characteristic diagnostic signs of the disease according to MRI and the treatment algorithms recommended today are also highlighted. The article summarizes data from foreign literature and our own clinical observations in order to develop an optimal protocol for MRI studies in patients with suspected hypophysitis, to develop recommendations for radiologists and endocrinologists for the correct results interpretation. The uniqueness of this review is the lack of data on the clinic, diagnosis and treatment of hypophysitis in the Russian literature today.

BACKGROUND: Cushing’s disease (CD) is a rare disorder of a persistent cortisol excess caused by ACTH-secreting pituitary tumor (corticotropinoma). Transsphenoidal surgery (TSS) is a treatment of choice for СD, which effectiveness range is from 70 to 90%. Recurrence rate after successful treatment is about 25%. If surgical treatment is unsuccessful or recurrence appear, radiation treatment is the next therapeutic option, which effectiveness range is also 90%, but the hypopituitarism rate as side effect of treatment is higher. Preoperative predictors of remission and recurrence are still unexplored what leads to further investigations.AIM: Analysis of remission and recurrence rates of pediatric CD after successful treatment according to preoperative MRI and therapeutic option.MATERIALS AND METHODS: We conducted a retrospective analysis of 90 pediatric patients with CD who were observed between 1992 and 2020 at the Endocrinology Research Centre.RESULTS: The most common clinical symptoms of CD were weight gain [94%] and growth retardation [72%]. Pituitary tumor was detected on radiological imaging in 53/90 patients [59%], there were no signs of visible adenoma in 37/90 of patients [41%]. 63 of 90 patients underwent TSS (70%), 27 patients underwent radiosurgery (30%). Remission rate after TSS was 71% [45/63], after radiosurgery — 85% [23/27]. There were no significant differences in remission rates after radical treatment according to preoperative MRI results (P=0.21 after TSS and P=0.87 after radiosurgery, х2 analysis). Recurrence after successful treatment was diagnosed in 10 patients. There were no significant differences in time to recurrence according to preoperative MRI results (P=0.055, х2 analysis). Time to recurrence was statistically different after TSS compared to radiosurgery (P=0.007, Kaplan–Meier analysis) and in the group with developed adrenal insufficiency in the early postoperative period (P=0.04, Kaplan–Meier analysis). Analysis of side effect of treatment showed that the frequency of growth hormone and gonadotrophin deficiency was statistically higher after radiosurgery (р<0.01, Kruskel–Wallis ANOVA test). Diabetes insipidus was diagnosed only after TSS. CONCLUSION: Results of our study didn`t allow to use MRI-results as predictor of effectiveness treatment in pediatric CD. Therapeutic option has an impact on time to recurrence, not on recurrence rates. The frequency of growth hormone and gonadotrophin deficiency was statistically higher after radiosurgery compared to TSS. Further studies are needed to identify predictors of remission and recurrence in CD.>< 0.01, Kruskel–Wallis ANOVA test). Diabetes insipidus was diagnosed only after TSS.CONCLUSION: Results of our study didn`t allow to use MRI-results as predictor of effectiveness treatment in pediatric CD. Therapeutic option has an impact on time to recurrence, not on recurrence rates. The frequency of growth hormone and gonadotrophin deficiency was statistically higher after radiosurgery compared to TSS. Further studies are needed to identify predictors of remission and recurrence in CD.

IV (XXVII) Национальный конгресс эндокринологов с международным участием «ИННОВАЦИОННЫЕ ТЕХНОЛОГИИ В ЭНДОКРИНОЛОГИИ» 22-25 сентября 2021 года МАГНИТНО-РЕЗОНАНСНАЯ ТОМОГРАФИЯ В ДИАГНОСТИКЕ ЭНДОКРИННОЙ ОФТАЛЬМОПАТИИ Бабаева Д .М ., Свириденко Н .Ю ., Бессмертная Е

12-13 июня 2021 г.ПРИМЕНЕНИЕ МАГНИТНО-РЕЗОНАНСНОЙ ТОМОГРАФИИ В ДИАГНОСТИКЕ ДИСПЛАЗИИ ЧЕРЕПА У ДЕТЕЙ С СИНДРОМОМ МАККЬЮНА-ОЛБРАЙТА-БРАЙЦЕВА Д.М..Бабаева, Н.В.Маказан, В.П.Владимирова, А

Despite the fact that pituitary adenomas are among the most frequent brain tumours, TSH-secreting pituitary adenomas (thyrotropinomas) are less than 1% of all adenomas. Due to the increase in the free fractions of thyroid hormones at normal or elevated TSH levels, the majority of patients with these pituitary adenomas have a long anamnesis of thyrotoxicosis which requires a differential diagnosis with thyroid pathology (Graves disease, toxic adenoma, autonomously functioning thyroid nodules). The diagnosis of the thyrotropinoma is quite challenging for clinicians. This article describes the case of a combination of the thyrotropinoma with primary hypothyroidism as a result of the Hashimotos disease. A feature of this article is the absence of a typical clinical picture of thyrotoxicosis in combination with an evaluated level of TSH on the background of constantly increasing substitution therapy for primary hypothyroidism. The picture of space-occupying lesion according to MRI of the brain allowed to suspect hormone-active pituitary adenoma (macroadenoma). As a result of surgical treatment (endonasal transsphenoidal adenomectomy), the level of TSH and free thyroid hormone levels were normalized in the postoperative period. The diagnosis of TSH-secreting pituitary adenoma was confirmed by histological and immunohistochemical analysis of postoperative material.

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.

Objective: To study MRI diagnostic possibilities in evaluating of kidneys and adrenals in individuals with arterial hypertension practicing active physical culture and sports. Design and method: MRI was performed in 29 males and 16 females. First group included 26 healthy volonteers (17–29 years old); second group included 19 individuals active physical culture and sports. (17–35 years old). Persons in both groups had normal parameters of blood examination, blood pressure, electrocardiogram, but after exercise stress demonstrated transitory arterial hypertension (AH) 155–170/95–105, and slight proteinyria. Standard MRI included T1, T2-weighted, fat saturation sequences, MRI with contrast enhancement Results: In 4 persons we found solitary serous cists of kidney. In 5 persons MRI revealed different kidney abnormities (aplasia, hypoplasia, dystopia, fused kidney). In 6 persons with normal hormonal parameters and absence of clinical signs, different kinds of adrenal glands pathology were found: adenomathosis (n = 4) and incidentalomas (n = 2, small, inactive tumors with size 9–12 mm). Conclusions: Thus, MRI is a high effective method of evaluation of kidneys and adrenals in individuals with arterial hypertension practicing active physical culture and sports, which is important to find clinically silent changes. These data are necessary in planning training regimen, in decision question of possibility to be involved in different kinds of sport, and prophylactic measures application.

Objective: To study MRI diagnostic possibilities in evaluating of brain and pituitary in individuals with hypertension practicing active physical culture and sports. Design and method: MRI was performed in 43 males and 27 females. First group included 44 individuals practicing active physical culture and sports (19 – 35 years old); second group included 26 healthy volunteers (18–30 years old). Persons in both groups had normal parameters of blood examination, blood pressure, electrocardiogram, but after exercise stress demonstrated transitory arterial hypertension (AH) 155–170/95–105. Standard MRI included flair-, T1-, T2-weighted images, and MRI with contrast enhancement. Results: We found that brain changes are more often in 1st than in 2nd group. Mild hydrocephaly (widening of subarachnoid spaces and ventricles) was found in 15 persons in 1st group (34%), and in 5 persons in 2nd group (19%). Arachnoid cysts were found in 8 persons (18%) in 1st group and in 1 person in 2nd group (4%). In 4 persons in 1st group we found signs of old hematomas (9%). In 3 persons of 1st group solitary cysts sized 6–12 mm of white matter were found (7%). In 1st group pituitary microadenomas were found in 2 women, diffuse pituitary hyperplasia - in 8 women, empty sella – in 4 women and 1 man. In 2nd group pituitary microadenomas were found in 1 women, diffuse pituitary hyperplasia - in 4 women, empty sella – in 1 women. Thus, we found pituitary changes: in 1st group - 34%, in 2nd group – 23%. In clinical status 32 persons of the 1st group marked headache during physical exercises. Some women with pituitary changes had increased prolactin level and menstrual disturbances. All these patients received appropriate treatment and dynamic examination. Conclusions: MRI is effective method of evaluation of brain and pituitary in individuals practicing active physical culture and sports, to find clinically silent changes. These data are important in finding clinically silent changes, planning training regimen and prophylactic measures application.

Оценивали распространенность TART среди 71 пациента с ВДКН в возрасте от 1 года до 19 лет и сравнивали методы визуализации (УЗИ и МРТ) TART. TART была выявлена в 14,1% случаев ВДКН (10 из 71; 1 пациент с простой вирильной и 9 - с сольтеряющей формой). Большинство TART (9 из 10) были выявлены с помощью УЗИ, МРТ позволило уточнить размеры образований. Обнаружена взаимосвязь между наличием TART и уровнями 17-ОНП и АРП на момент обследования, количеством определений и уровнем АРП в течение последнего года. TART чаще возникает у детей с плохо контролируемым лечением: недостаточность как глюко-, так и минералокортикоидов является предрасполагающим, но не единственным фактором риска развития этого заболевания.