Hypoparathyroidism is an endocrine disease characterized by reduced production of parathyroid hormone by the parathyroid glands or tissue resistance to its action. This is accompanied by disturbances in phosphorus-calcium metabolism. The main cause of hypoparathyroidism is damage or removal of the parathyroid glands during surgery on the neck organs. Autoimmune hypoparathyroidism is the second most common form of the disease, which occurs, as a rule, within the autoimmune polyglandular syndrome type 1. The development of chronic hypoparathyroidism of any etiology requires lifelong appointment of multicomponent therapy, as well as careful monitoring and an individual approach. In the absence of adequate dynamic monitoring, multiple complications develop from vital organs, in particular calcification of the urinary system, soft tissues and the brain; cardiovascular pathology; visual disturbances; muscle and bone diseases leading to decreased quality of life of patients. Thus, timely diagnosis, rationally selected drug therapy and competent patient management will reduce the risks of complications, improve the prognosis, and reduce the frequency of hospitalizations and disability of patients with chronic hypoparathyroidism. The article presents the main recommendations of the new project of clinical guidelines for patients with hypoparathyroidism, approved by the Ministry of Health of the Russian Federation in 2021. They include the algorithms of diagnosis, treatment and dynamic monitoring of hypoparathyroidism, as well as management of postsurgical hypocalcemia and medical care of and the disease during pregnancy.
Hyperparathyroidism is a syndrome characterized by an excessive secretion of parathyroid hormone. Etiologically, hyperparathyroidism is subdivided into primary hyperparathyroidism, which develops as a result of parathyroid adenoma, carcinoma or hyperplasia, and secondary hyperparathyroidism, which happens as a compensatory response to a hypocalcemia caused by condition outside the parathyroid glands. Turner syndrome may also be accompanied by mineral metabolism disorders of various etiology. An association of hyperparathyroidism and Turner syndrome is interesting because of multifactorial impact on bone mineral density, but only few cases of such coexistence have been previously described in the literature. This article describes two patients with Turner syndrome and hyperparathyroidism of different etiology. Hyperparathyroidism, normocalcemia, vitamin D deficiency, osteoporosis, parathyroid tumors were found in both cases. In one case a number of assays was performed to confirm the patient’s normocalcemic primary hyperparathyroidism, and surgery was performed to achieve remission. In the second case, treatment of vitamin D deficiency resulted in normalization of serum concentration of parathormone, after which the patient was prescribed antiresorptive therapy. The pathogenetic association between Turner syndrome and hyperparathyroidism requires further investigation. Comprehensive approach to the diagnosis and treatment of mineral metabolism disorders are essential for patients with coexistence of these two diseases.
One have described the clinical case of severe course of Trichinella infection. It has revealed some difficulties in diagnosis due to polymorphism of clinical picture, affection of different organs and systems by parasite. This demands the specialists of different profiles to be altered to this parasitic infection.
IntroductionUntil recently no major epidemiological research of primary hyperparathyroidism (PHPT) has been conducted in the Russian Federation, this led to the creation of the Russian online registry. The objective of this study is to estimate the clinical and biochemical profile, classical and non-classical complications, surgical intervention and medical therapy of the patients with different forms of PHPT in the Russian Federation.Materials and methodsThe cross-sectional, observational, continuous study was conducted at the Endocrinology Research Centre (Moscow). The present study explored retrospective data from 6003 patients submitted to the Registry between 12.12.2016 and 25.10.2022 from 81 regions of the Russian Federation (http://pgpt.clin-reg.ru/).ResultsThe median age was 59 [60; 66] years with a female:male ratio of 11.7:1. Symptomatic PHPT was observed in 74.3% while asymptomatic form - only in 25.7% of cases. Bone pathology was the predominant clinical manifestation in 62.5% of cases (n=2293), mostly in combination with visceral complications 45.7% (n=1676). The majority of patients (63.3%) had combined visceral disorders including kidney damage in 51.8% and gastroduodenal erosions/ulcers in 32.3% of patients. Symptomatic patients were older (60 [53; 67] vs. 54 [45; 62] years, p<0.001) and had more severe biochemical alterations of calcium-phosphorus metabolism. Cardiovascular disease (СVD) was recorded in 48% of patients, among them the most frequent was arterial hypertension (up to 93.9%). A genetic test was conducted in 183 cases (suspicious for hereditary PHPT) revealing the mutations in MEN1, CDC73, RET genes in 107, 6 and 2 cases, respectively. Surgery was performed in 53.4% of patients with remission achievement in 87%, the relapse/persistence were recorded in 13% of cases. Histological examination revealed carcinoma in 4%, atypical adenoma in 2%, adenoma in 84% and hyperplasia in 11% of cases. Drug therapy was prescribed in 54.0% of cases, most often cholecalciferol.ConclusionThe detection rate of PHPT has increased in the Russian Federation in recent years. This increase is associated with the start of online registration. However, the majority of patients remain symptomatic with significant alterations of phosphorus-calcium metabolism that indicates delayed diagnosis and requires further modifications of medical care.
IntroductionCardiovascular diseases (CVD) and metabolic disorders (MD) have retained leading positions in the structure of morbidity and mortality for many years. Primary hyperparathyroidism (PHPT) is also associated with a greater incidence of CVD and MD. The aim of the present study was to describe the prevalence and structure of CVD and MD in hospitalized patients with PHPT and to search for possible associations between these pathologies.Methods838 patients with a verified PHPT were included in the study. The studied cohort was divided into 2 groups according to their age at the time of admission: patients aged 18 to 49 years (group A, n = 150); patients aged 50 years and older (group B, n = 688).ResultsThere were no significant differences between two groups in parameters of calcium-phosphorus metabolism. Obesity was diagnosed in 24.2% of patients in group A and in 35.9% in group B. Type 2 diabetes mellitus was more common in older patients (14.4% in group B vs. 2.6% in group A). Arterial hypertension, ischemic heart disease, chronic heart failure and brachiocephalic arteries atherosclerosis were more frequent in older patients, occurring in 79.1%, 10.8%, 8.4%, and 84% of cases respectively. The cutoff points that increased the risk of CVD detection turned out to be age above 56 years, eGFR below 92 ml/min/1.73m2, BMI above 28.3 kg/m2.DiscussionThe present study demonstrated a high incidence of some CVD, as well as disorders of lipid, carbohydrate and purine metabolism in patients with PHPT.
The high prevalence of COVID-19 requires the research progress on the disease pathogenesis. There is a lot of data confirming the association between mineral metabolism and the severity of COVID-19.AIM:To study the dynamics of mineral metabolism parameters in patients with a confirmed COVID-19 at the time of hospitalization and after discharge, including the impact of etiotropic and pathogenetic therapy on them.MATERIALS AND METHODS:A single-center study of 106 patients (aged ≥18 years) with clinically or laboratory confirmed diagnosis of COVID-19 was carried out at the Endocrinology Research Centre, Moscow. Baseline biochemical parameters, including serum calcium, phosphorus, albumin, 25(OH)D, parathyroid hormone (PTH), inflammatory markers, and instrumental assessment of COVID-19 severity were performed before specific immunotherapy, as well as on 3rd and 7th days of hospitalization and before discharge. Statistical analysis was performed with Statistica 13 software (StatSoft, USA).RESULTS:On the first day, hypocalcemia (low albumin-adjusted calcium level) was detected in 40.6% of cases, the prevalence of vitamin D deficiency/insufficiency amounted to 95.3% of cases. At the same time, secondary hyperparathyroidism was identified only in 14.2% of patients. A comparative analysis of mineral metabolism during hospitalization (between 1, 3, 7 days of hospitalization and before discharge) during baricitinib treatment revealed a statistically significant increase in albumin-adjusted calcium by the end of hospitalization (p<0.001, Friedman criterion, Bonferroni correction p0=0.01). A pairwise comparison of subgroups, depending on the therapy, revealed a statistically significantly lower level of albumin-adjusted calcium on 3rd day among patients on baricitinib monotherapy or combined with tocilizumab compared with a subgroup of patients undergoing etiotropic treatment (2.16 [2.13; 2.18] mmol/l vs 2.23 [2.19; 2.28] mmol/l, p=0.002, U-test, Bonferroni correction p0=0.012).CONCLUSION:Patients with severe coronavirus infection are characterized by a high prevalence of vitamin D deficiency and hypocalcemia. Associations between calcium and saturation as well as the severity of lung lesion characterizes hypocalcemia as an important predictor of severe course and poor outcome in COVID-19. Pathogenetic therapy with baricitinib, including in combination with tocilizumab, contributes to achieve normocalcemia, but further studies are required.
По данным ВОЗ пожилые люди, а также больные с сопутствующими, в том числе эндокринными за- болеваниями подвержены наибольшему риску тяжелого течения коронавирусной инфекции (COVID-19). Ученые и клиницисты всего мира активно изучают особенности патогенеза COVID-19 и его влияние на эн- докринную патологию. ЦЕЛЬ: изучить структуру эндокринной патологии, а также особенности минерального обмена у паци- ентов, госпитализированных с COVID-19. МАТЕРИАЛЫ И МЕТОДЫ: проведено одноцентровое ретроспективное исследование 193 пациентов. В первый день госпитализации до назначения упреждающей терапии было выполнено расширенное ла- бораторное обследование. Сравнение независимых групп по качественным признакам выполнено с по- мощью критерия Хи-квадрат Пирсона (χ2). Исходный критический уровень значимости (p) при проверке статистических гипотез принимался равным 0,05. РЕЗУЛЬТАТЫ: среди обследованных пациентов выявлены следующие эндокринопатии: СД (17,6%), ожирение (43,5%), узловой зоб (5,1%), ПГПТ (0,5%), ВГПТ (38,9%). Дефицит/недостаточность витамина D диагностированы в 96,8%. Значимых отклонений кальцитонина не зафиксировано (медиана 1 [1; 1] пг/мл). Медиана 25(ОН)D составила 12,10 [6,98; 17,00] нг/мл, ПТГ - 43,02 [29,51; 58,53] пг/мл, альбумин-скорректиро- ванного кальция 2,18 [2,11; 2,26] ммоль/л. Гипокальциемия зафиксирована в 38,9% случаев, в то время как гиперкальциемия в 1% (n=2). ПТГ имел тенденцию к умеренной отрицательной корреляции с лабораторно подтвержденным COVID-19 (r= -0,211, p=0,003); отмечена умеренная положительная корреляция альбу- мин-скорректированного кальция с лейкоцитами (r=0,297, p<0,001), с тромбоцитами (r=0,307, p<0,001). Гипокалиемия имела умеренную отрицательную связь с высоким уровнем СРБ (r= -0,211, р=0,003) и ЛДГ (r= -0,213, р=0,003). В подгруппе тяжелой степени тяжести были ниже уровни 25(ОН)D, общего кальция и альбумина (р<0,05). При пошаговом логистическом регрессионном анализе была выявлена ассоцииация гипоальбуминемии с более высокой степенью поражения легких по МСКТ (ОШ 0,815, 95% ДИ 0,736 —0,903), а также с более высокими значениями D-димера (ОШ 0,864, 95% ДИ 0,773-0,967). Гипокалиемия коррелировала с повы- шенными значениями ЛДГ (ОШ 0,175, 95% ДИ 0,056—0,545) и D-димера (ОШ 0,101, 95% ДИ 0,022-0,457). При сравнении подгруппы с эндокринными заболеваниями (n=139) с подгруппой без эндокринных заболеваний (n=54) в первой отмечены более низкие уровни альбумина (р=0,018, Σ2), магния (л, р=0,056, Σ2), рСКФ (р=0,0003, Σ2), а уровни ФРФ-23 и D-димера были более высокими (р=0,009 и р=0,055, соответ- ственно, Σ2). Также, у них чаще развивался «цитокиновый шторм» (48,1% против 30,4%, р=0,021, Σ2). Для пациентов с СД (n=34) были характерны более высокие маркеры воспаления, статистически значимые для СРБ, ИЛ-6, ИЛ-1β (p<0,05), и более низкий уровень IgG (р=0,034). Кроме того, в подгруппе с СД чаще встречались гипоальбуминемия, гипомагниемия и недостаточность витамина D (р<0,05) и была характерна высокая частота «цитокинового шторма» (51,3% против 31,4%, р=0,0203, Σ2) с летальным исходом (15,4% против 5,8%, р=0,047, Σ2). ВЫВОДЫ: высокая частота эндокринной патологии и ее взаимосвязь с тяжелым течением COVID-19 диктует необходимость расширенного обследования во время госпитализации. Своевременная диагно- стика и коррекция выявленных нарушений нужна для улучшения прогноза пациентов.
ЦЕЛЬ: представление серии случаев симптомного первичного гиперпаратиреоза (ПГПТ) во время беременности. МАТЕРИАЛЫ И МЕТОДЫ: представлена серия из 3 случаев ПГПТ, диагностированного во время беременности, с различными вариантами ведения и клиническими исходами. РЕЗУЛЬТАТЫ: 1. Пациентка Д., 35 лет. ПГПТ диагностирован на 23 неделе беременности (ПТГ 232,7 пг/мл, альбумин-скорректированный кальций (Са) 3,0 ммоль/л, гиперкальциурия 14,8 ммоль/сут), при поступлении к ведущим жалобам относились сухость во рту, утомляемость. Генетическое исследование не выявило мутаций, ассоциированных с ПГПТ. После визуализации при УЗИ образования левой нижней околощитовидной железы (ОЩЖ) проведена селективная паратиреоидэктомия (ПТЭ) на 26 неделе гестации с достижением ремиссии (ПТГ 45 пг/мл, Са 2,28 ммоль/л), в послеоперационном периоде верифицирована аденома ОЩЖ. Роды срочные протекали без осложнений у матери и ребенка. 2. Пациентка С., 35 лет с длительным предшествующим анамнезом нефролитиаза и множественных низкоэнергетических переломов костей конечностей. ПГПТ диагностирован на 29 неделе беременности (ПТГ 55,5 пмоль/л, Са 3,28 ммоль/л), при УЗИ визуализировано образование левой нижней ОЩЖ 3,3х2,5х1,8 см. По решению врачебного консилиума с учетом тяжести гиперкальциемии рекомендован прием цинакальцета 30-60 мг/сут с последующей ПТЭ в максимально короткие сроки, однако от предложенного лечения пациентка отказалась. Кесарево сечение выполнено на 38 неделе гестации, со слов пациентки, без специфических осложнений. В течение 1,5 лет проводилось грудное вскармливание, за этот период произошел перелом левой плечевой кости с проведением остеосинтеза. Через 2 года после родов при поступлении в специализированный центр ПТГ 1044 пг/мл, Са 2,9 ммоль/л, кальциурия 13,3 ммоль/сут, выявлены двусторонний нефролитиаз, остеопороз с множественными переломами грудных позвонков, фиброзно-кистозный остит. Выполнена успешная левосторонняя ПТЭ (ПТГ 50,1 пг/мл, Са 2,18 ммоль/л), морфологически диагностирована карцинома ОЩЖ. 3. Пациентка Н., 32 года. ПГПТ диагностирован на сроке 18-19 недель при наличии рвоты до 6 раз в сутки, заторможенности, снижении веса (ПТГ 226,4 пг/мл, Са 3,31 ммоль/л). Учитывая срок беременности, угрозу гиперкальциемического криза рекомендовано хирургическое лечение, в дооперационном периоде по решению врачебной комиссии назначен цинакальцет 30 мг/сут. На сроке 22 недели гестации при госпитализации в ФГБУ «НМИЦ эндокринологии» Минздрава России на фоне приема терапии ПТГ 91,7 пг/мл, Са 2,76 ммоль/л, кальциурия 2,9 ммоль/сут. После лечения эрозивного гастрита выполнено радикальное удаление аденомы левой нижней ОЩЖ (ПТГ 13 пг/мл, Са 2,49 ммоль/л). Беременность закончилась своевременными родами путем кесарева сечения без осложнений. ВЫВОДЫ: в настоящее время ПТЭ во втором триместре беременности является оптимальным выбором для пациенток с симптомным ПГПТ и выраженной гиперкальциемией. Наблюдение рационально проводить лишь в случаях бессимптомного течения заболевания. Специфическая медикаментозная терапия ПГПТ во время беременности возможна по решению врачебной комиссии, так как остается до настоящее времени назначением «off-label».
ЦЕЛЬ: представление серии случаев гиперпаратиреоза различного генеза при синдроме ШершевскогоТернера (СШТ). Клинический случай 1: пациентка А. обратилась в НМИЦ эндокринологии в 2019 г. в возрасте 31 года с жалобами на ломоту в костях. В возрасте 13 лет у пациентки был диагностирован СШТ (46Хi/45Х0), постоянно получала гормональную заместительную терапию. В 23 года впервые отмечена гиперкальциемия, повышение паратгормона (ПТГ), тогда же при УЗИ обнаружено образование левой нижней околощитовидной железы (ОЩЖ) 14х8мм. При последующем обследованиина фоне нормокальциемии (Са скорр. 2,47 ммоль/л, 2,15-2,55), нормофосфатемии (0,97 ммоль/л, 0,74-1,52), нормокальциурии (6,6 ммоль/сут, 2,5-8,0) и недостаточности витамина D (20 нг/мл) отмечалось повышение ПТГ до 151 пг/мл. После достижения целевого уровня 25(ОН)D проведена проба с альфакальцидолом в максимальной дозе 1,5 мкг/сут - без динамики. Проведена проба с кальцитриолом, при сохранении нормокальциемии и повышения ПТГ до 130 пг/мл развилась гиперкальциурия, диагностирован первичный гиперпаратиреоз (ПГПТ). Из осложнений ПГПТ у пациентки имелся нефролитиаз, эрозивный эзофагит, снижение минеральной плотности костной ткани (МПК) в шейке бедренной кости до -2.5 SD по Z-критерию. Клинических данных за синдром множественных эндокринных неоплазий не получено. После топической диагностики проведена селективная паратиреоидэктомия, достигнута нормализация ПТГ (20 пг/мл) при гипокальциемии (Са общ. 2,03 ммоль/л). Гистологически верифицирована аденома ОЩЖ. Клинический случай 2: пациентка Д., 47 лет обратилась в НМИЦ эндокринологии с жалобами на общую слабость. С 15 лет наблюдалась по поводу СШТ, ГЗТ не получала, в 2018 г. на фоне нормокальциемии (Са скорр. 2,3 ммоль/л), 25(ОН)D 81 нг/мл и сохранной СКФ отмечено повышение ПТГ до 180 пг/мл (15-65). В ходе дообследования отмечено повышение ПТГ до 147 пг/мл на фоне нормокальциемии (Са скорр. 2,47 ммоль/л), нормофосфатемии (0,95 ммоль/л), нормокальциурии (2,87 ммоль/сут), выраженного дефицита витамина D (6,8 нг/мл). При УЗИ визуализированы образования правой и левой нижних околощитовидных желез (до 0,8 см и 1,9 см соответственно). У пациентки также выявлено снижение МПК до -5,5 SD по Т-критерию в лучевой кости, нефролитиаз. Назначен колекальциферол в насыщающей дозе, на фоне чего ПТГ снизился до 113 пг/мл при сохранении нормокальциемии и гипокальциурии, что в большей степени соответствовало проявлениям вторичного гиперпаратиреоза. Рекомендована поддерживающая терапия колекальциферолом, динамический контроль с последующим решением вопроса о проведении иных дифференциально-диагностических проб. ВЫВОДЫ: СШТ может маскировать сопутствующую патологию минерального обмена за счет ожидаемого, обусловленного дефицитом эстрогенов снижения МПК. В то же время, указанное состояние требует тщательной дифференциальной диагностики с иными нарушениями минерального обмена (в частности, ПГПТ), в том числе, у молодых пациенток.
Методическое пособие по вторичному гиперпаратиреозу вследствие хронической болезни почек 3 ДОРОГИЕ КОЛЛЕГИ!Минеральные и костные нарушения, сопутствующие хронической болезни почек -это многофакторное прогрессирующее заболевание, наиболее распространенным и тяжелым проявлением которого является вторичный гиперпаратиреоз.Пациенты с вторичным гиперпаратиреозом вследствие хронической болезни почек нуждаются в постоянном наблюдении, регулярном обследовании, проведении профилактических и лечебных мероприятий, поэтому настоящее пособие разработано для широкого круга специалистов -не только для эндокринологов, но и нефрологов, урологов, терапевтов, врачей общей практики, кардиологов и др.Коллектив авторов постарался в доступной форме предоставить обзор последних научных данных о патогенезе, клинических проявлениях, методах лабораторной и инструментальной диагностики заболевания.Особое внимание при подготовке материала было уделено темам, вызывающим наибольшее количество вопросов -выбору и режимам дозирования лекарственных препаратов, их комбинациям, показаниям к хирургическому лечению, объему операции
BACKGROUND: In patients with end-stage CKD, receiving renal replacement therapy (RRT) with programmed hemodialysis (HD), the severity of complications is associated with metabolic disturbances: accumulation of uremic toxins, nephrogenic anemia, secondary hyperparathyroidism (SHPT), extraskeletal calcification, impaired clearance and rhythm of hormone secretion.AIM: To evaluate the main biochemical and hormonal parameters, and manifestations of mineral bone disease (MBD) in patients receiving RRT with HD, before and after hemodialysis, taking into account the presence or absence of diabetes mellitus.MATERIALS AND METHODS: We divided all patients receiving RRT with HD in two groups: #1 (n=24) — patients with DM, #2 (n=16) — patients without DM. All of them had their blood analyzed before and immediately after the HD. Data analysis was performed with the Statistica 13 (StatSoft, USA). A prognostically significant model was considered at p<0.05.RESULTS: The level of iPTH, both at baseline and after HD, was lower in group #1 (p<0.001). The level of alkaline phosphatase (AP) was significantly higher in group #2 (p=0.012). In both groups before HD, a high incidence of hypocalcemia was detected (according to albumin-corrected calcium in group #1 in 58.3%, in group #2 in 43.7% of cases, p = 0.366) and hyperphosphatemia (in 66.7% and in 43 .7% of cases, respectively, p=0.151). Hypocalcemia after HD in group #1 persisted in 14%, in group #2 — in 20% of cases (p>0.05); hyperphosphatemia in group #1 was completely leveled, in group #2 it persisted in 7% of cases (p=0.417). Prior to the HD session, group #1 had significantly higher levels of RAGE, glucagon, immunoreactive insulin (IRI), cortisol, and glucose than after the HD session (p<0.05). In group #2, after HD, the levels of glucagon, IRI and cortisol significantly decreased (p<0.05), and the level of 3-nitrotyrosine (3-HT) increased significantly (p=0.026). In group #1, fibrocalcinosis of the heart valves according to ECHO and calcification of the arteries of the lower extremities according to ultrasonic doplerography were more common than in group #2 (42% vs 25%, p<0.001 and 75% vs 37.5%, p=0.018, respectively). (χ2)). Compression fractures occurred with the same frequency in both groups (60%). A decrease in bone mineral density (BMD) to the level of osteopenia was noted more often in group #1 (50% vs 18.8%), and osteoporosis was more common in group #2 (68.8% vs 33.3%) (p<0.001, χ2).CONCLUSION: The low level of PTH in group #1 may reflect the effect of diabetes on calcium-phosphorus metabolism. Patients with DM have an increased risk of renal osteodystrophy with a low bone turnover because of a number of metabolic factors inherent in diabetes. At the same time, the dynamics of phosphorus and calcium indicators during the HD procedure were similar.
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.
XXVIII) НАЦИОНАЛЬНЫЙ ДИАБЕТОЛОГИЧЕСКИЙ КОНГРЕСС С МЕЖДУНАРОДНЫМ УЧАСТИЕМ «САХАРНЫЙ ДИАБЕТ И ОЖИРЕНИЕ -НЕИНФЕКЦИОННЫЕ МЕЖДИСЦИПЛИНАРНЫЕ ПАНДЕМИИ XXI ВЕКА» СБОРНИК ТЕЗИСОВ 122 МИНЕРАЛЬНЫЕ И КОСТНЫЕ НАРУШЕНИЯ У ПАЦИЕНТОВ ДО И ПОСЛЕ ЗАМЕСТИТЕЛЬНОЙ ПОЧЕЧНОЙ ТЕРАПИИ ПРОГРАММНЫМ ГЕМОДИАЛИЗОМ Маганева И .С
IntroductionChronic hypoparathyroidism is a relatively rare disease associated with multicomponent medical therapy and various complications. The analysis of large databases of patients with chronic hypoparathyroidism is a necessary tool to enhance quality of medical care, as well as to determine the optimal clinical and therapeutic approaches, and prognostic markers of the disease.The Aimof this study is to estimate the clinical and biochemical profile, long-term complications, medical therapy and disease control of the patients with chronic postsurgical and non-surgical hypoparathyroidism.Materials and Methodsthe cross-sectional, observational, continuous study was based on the Russian Registry of patients with hypoparathyroidism. 544 patients from 63 regions of the Russian Federation were included in this study.ResultsThe majority of cases had postsurgical etiology (88.4%). Postsurgical hypoparathyroidism prevailed in females (р<0.001). About a half of patients had blood calcium and phosphorus targets, 56 and 52% respectively. Nephrolithiasis was confirmed in 32.5%, nephrocalcinosis - in 12.3% of cases. The risk of nephrocalcinosis/nephrolithiasis increased by 1.85 times with disease duration more than 4.5 years. The cataract was found in 9.4%. The cut-off point for the development of cataracts was 9.5 years, with a 6.96-fold increased risk. The longer duration of hypoparathyroidism of any etiology was associated with more frequent cataract (p=0.0018).We found brain calcification in 4%, arrhythmias in 7.2% and neuropsychiatric symptoms in 5.15% of cases. Generally, the BMD in the studied group corresponded to age values, and there was no evidence for the phenomenon of high bone density. TBS was consistent with normal bone microarchitectonics. In our study, the majority of patients (83.5%) was treated with standard therapy of calcium and vitamin D supplements. 5 patients with severe disease course were treated with rhPTH (1–34).ConclusionsAnalysis of the presented database indicates insufficient diagnosis of the complications associated with chronic hypoparathyroidism. Overall, hypoparathyroidism is associated with higher risks of renal stone formation, decreased GFR, cataract especially in patients with longer duration of disease.
СРАВНИТЕЛЬНЫЙ АНАЛИЗ ПОКАЗАТЕЛЕЙ УГЛЕВОДНОГО ОБМЕНА И КОНТРИНСУЛЯРНОГО ОТВЕТА У ПАЦИЕНТОВ, НАХОДЯЩИХСЯ НА ЗАМЕСТИТЕЛЬНОЙ ПОЧЕЧНОЙ ТЕРАПИИ
Background and objective:Preoperative diagnosis of parathyroid carcinoma (PC) is critical for the determination of the scope of surgical intervention. Nowadays, specific diagnostic markers for differentiation of PC and benign tumors are unknown, and less than half of patients with PC undergo necessary en bloc surgery. The aim of this study was to develop the instrument for preoperative diagnosis of PC. Methods:A multi-center retrospective study included 242 patients with primary hyperparathyroidism: 50 patients with PC, 30 with аtypical adenoma (AA), and 162 with adenoma of the parathyroid glands. Results:Patients with PC and AA had higher levels of PTH, ionized and albumin-corrected calcium, ALP, volume and the largest diameter of neoplasm, and the higher frequency of GFR decrease less than 60 ml/min/1.73 m2 compared to patients with adenoma. The frequency of low-energy fractures was higher in the carcinoma group versus the adenoma group (32% vs 8%). Heterogeneous structure and indefinite contour of glands detected by US were more typical for PC than for AA and adenomas. The mathematical model was developed using CatBoost gradient boosting algorithm for the noninvasive preoperative differential diagnosis of PC, AA, and adenoma. Conclusions:Model can predict adenoma with PPV 100% and PC with PPV 81-92%. Using model clinicians could plan extended en bloc resection for PC and selective parathyroidectomy for adenoma. If AA is predicted, he has to make a decision on the choice of the necessary volume of PTE based on his experience, because AA are the zone of uncertainty.
BACKGROUND:Vitamin D (25-hydroxyvitamin D [25(ОН)D]) deficiency (<20 ng/mL) and insufficiency (20-29 ng/mL) are common in primary hyperparathyroidism (PHPT), but data regarding the vitamin D metabolism in this population is limited.AIM:The aim of this study is to estimate the vitamin D metabolites and their relationship with the main parameters of phosphorus-calcium metabolism in patients with PHPT at baseline and on the background of a single dose of cholecalciferol 150,000 IU.MATERIALS AND METHODS:A single-center interventional, dynamic, prospective, comparative study has been carried out. The study included 54 participants, divided into two groups: the 1st group included 27 patients with confirmed PHPT, the 2nd control group (n = 27), matched on gender (p = 0.062). The study included 4 visits; the baseline laboratory examination and a bolus dose of cholecalciferol were performed at the visit 1, the subsequent visits included a dynamic laboratory examination.RESULTS:Vitamin D deficiency (<20 ng/ml) was detected in 69% of patients with PHPT. In the PHPT group (before cholecalciferol therapy), there was a direct association of 1.25(OH)2 D3 with albumin-corrected and ionized calcium, as well as between the 25(OH)D3 /24.25(OH)2 D3 ratio with PTH and magnesium. After taking of cholecalciferol, the levels of 1.25(OH)2 D3 and 25(OH)D3 /24.25(OH)2 D3 were significantly increased, and the levels of 25(OH)D3 /1.25(OH)2 D3 were significantly declined at all visits among patients with PHPT. The common 25(OH)D level was comparable to the control group, however the levels of 1,25(OH)2 D3 in patients with PHPT were 55% higher at baseline, and after taking of cholecalciferol 150,000 IU. They remained increased by 3-7 days by an additional 23-36%, significantly higher than those in the control group: 44%, 74% and 65%, at visits 2, 3 and 4, respectively (p<0.05). The taking of 150,000 IU cholecalciferol in the PHPT group did not lead to a significant increase in hypercalcemia and hypercalciuria, which indicates the safety of this dose in patients with mild hypercalcemia (albumin corrected calcium <3 mmol/l). None of the study participants experienced any side effects.CONCLUSION:The completely comprehensive assessment of vitamin D metabolites was carried out for the first time in patients with PHPT before and after using a bolus dose of cholecalciferol. The results confirmed the differences of vitamin D metabolism in chronic excessive secretion of PTH compared to control group, which is new data in the pathogenesis of the disease, and can be used to develop optimal regimens for cholecalciferol taking in this population.