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

Background. Primary hyperparathyroidism (PHPT) is an endocrine disease, due to the formation of excess parathyroid hormone secretion and a disturbance of phosphorus-calcium metabolism. PHPT is associated with reduced renal function and related increased mortality, therefore renal function impairment defined as a glomerular filtration rate (GFR) less than 60 mL/ min/1.73 m 2 is considered as an indication for surgical treatment. Some patients with PHPT have secondary cardio-renal syndrome (type 5), which is characterized by the presence of combined renal and cardiac pathology. THE AIM: Evaluate the GFR in patients with mild and symptomatic PHPT and its changes after parathyroidectomy and on medical treatment. Patients and methods : A cross-sectional comparative and prospective study was conducted on 100 patients with confirmed PHPT. A clinical and laboratory examination was performed at 6, 12, and/or 24 months and included an assessment of the PHPT complications as well as expression of cardio-renal syndrome according to the selected management tactics. Changes in GFR were evaluated in 29 patients after surgical treatment and in 27 patients on conservative management. Glomerular filtration rate was calculated by the Modification of Diet in Renal Disease Study (MDRD) formula. The chronic kidney disease (CKD) stage was estimated accordingly to current recommendations. Results: We included 100 patients (10 men and 90 women) with PHPT: 33 with mild and 67 with symptomatic PHPT. The median age was 57 years [51;61]. Mean GFR was 84,1 [73,3; 97,9] ml/ min/1,73 m 2 . Prevalence of CKD 3-4 in patients with PHPT was recorded at 11 %. Patient management tactics were determined in accordance with international recommendations. In the long-term period after surgery, we observed the decrease of GFR in patients with mild form within the limits of CKD 1-2 (p=0,013, W-test). No significant changes in GFR were noted during medical management and monitoring. Summary, the cardio-renal syndrome (CKD of any stage and cardiovascular pathology) was revealed in 26 % of patients. Arterial hypertension was registered in 40 %. Conclusions : We found a high frequency of renal function impairment in patients with PHPT, including patients with a mild form of the disease. We did not observe any significant improvement in renal filtration function, after surgical treatment and remission of PHPT. The safety of conservative tactics has been confirmed. The high frequency of cardiovascular pathology in patients with PHPT is beyond doubt and requires prospective studies in the larger cohorts of patients.

There are no large-scale epidemiological studies on primary hyperparathyroidism (PHPT) in Russia. The high prevalence of the disease, the high risk of disability and death in this cohort of patients requires the study of the epidemiological and clinical structure of PHPT to determine the extent of medical care.Evaluate the frequency of PHPT detection and characterize its clinical forms in Russia using an online registry.The object of the study is the database of the State Register of Patients with PHPT 1914 patients from 71 regions of the Russian Federation. New cases of the disease, as well as dynamic indicators are recorded when patients visit outpatient clinics or medical institutions. The analysis of data made at the end of December 2017 was carried out. The following parameters were evaluated: demographic and clinical indicators; indicators of phosphorus-calcium metabolism, the main forms of PHPT and its course, the primary characteristic of PHPT in hereditary syndromes and parathyroid carcinoma. Results are presented as mean and standard deviations, or medians and quartiles; descriptive statistics of qualitative attributes absolute and relative frequencies.the total number of patients with PHPT in the registry on 31 of December 2017 was 1914 cases (0.001% of the population of the Russian Federation). Identification of PHPT was 1.3 cases per 100 thousand of the population in Russia, 7.6 cases in Moscow, 6.1 cases per 100 thousand in the Moscow region. The average age of patients at the time of diagnosis was 55.6 10 years. The active phase of the disease was registered in 84.6% of patients (1620/1914), most of whom had a symptomatic PHPT 67.1% (1087/1620), and 32.9% a asymptomatic disease (533/1620). Symptomatic disease with visceral complications was detected in 15.8% cases (172/1087), with bone complications in 48.4% (526/1087). The mixed form of the disease was detected in 35.8% of patients with manifest form (389/1087). Normocalcemic variant PHPT (nPHPT) was registered in 14.5% cases (234/1620). Sporadic PHPT occurs in 83% of cases (1592/1914). 326 patients (17%) had a suspicion for hereditary form of the disease: average age was 31.2 12.3 years. A genetic analysis was conducted in 61 patients (3.2%): showed the mutation in the MEN1 gene in 2.9% of cases (55/1914) and the mutation in the CDC73 gene in 0.3% of cases (6/1914) (HPT-JT syndrome). Parathyroid carcinoma was confirmed in 1.8% of all patients (35/1914). Surgical treatment was performed in 64.5% of patients (1234/1914). Remission was achieved in 94% of cases (1160/1234), in 6% of cases relapse after surgical treatment or persistence of PHPT was recorded.detection of PHPT in the Russian Federation raised in comparison to 2016, which is associated with an active start of registration of patients in the regions. At this stage, it is necessary to modify the principles of registration and control, to make a platform for gathering information and calculating the necessary volumes of medical care for PHPT patients.Обоснование. В России отсутствуют широкомасштабные эпидемиологические исследования по первичному гиперпаратиреозу (ПГПТ). Широкая распространенность заболевания, а также повышение риска инвалидизации и смерти у данной когорты пациентов диктуют необходимость изучения эпидемиологической и клинической структуры ПГПТ для определения объема медицинской помощи. Цель: оценить распространенность ПГПТ и охарактеризовать его клинические формы в России с помощью онлайн регистра. Методы. Объектом исследования является база данных регистра пациентов с ПГПТ 1914 пациентов из 71 региона РФ. Проведен анализ данных, внесенных на конец декабря 2017 г. Оценены следующие параметры: демографические и клинические показатели, показатели фосфорно-кальциевого обмена, основные формы ПГПТ и его течение, представлена первичная характеристика ПГПТ в рамках наследственных синдромов и злокачественного поражения околощитовидных желез. Результаты представлены средними и среднеквадратичными отклонениями, либо медианами и квартилями, описательная статистика качественных признаков абсолютными и относительными частотами. Результаты. Общая численность пациентов с ПГПТ в регистре на 31.12.17 составила 1914 человек (0,001% населения РФ). Выявление ПГПТ на 100 тыс. населения составляет по Российской Федерации 1,3 случая, в Москве 7,6 случая, в Московской области 6,1 случая. Средний возраст пациентов на момент установки диагноза составил 55,610 лет. Активная фаза заболевания зарегистрирована у 84,6% (1620/1914) пациентов, большая часть которых имела манифестное течение ПГПТ 67,1% (1087/1620), а 32,9% (533/1620) мягкое. При манифестном течении смешанная форма заболевания выявлена у 35,8% (389/1087) пациентов; изолированная костная форма у 48,4% (526/1087), изолированная висцеральная у 15,8% (172/1087). Нормокальциемический вариант ПГПТ (нПГПТ) на данный момент составляет 14,5% случаев (234/1620) (ранее 9%). Спорадический ПГПТ встречается в 83% (1592/1914) случаев. Подозрение на наследственную форму ПГПТ имеется у 326 (17%, 326/1914) пациентов: средний возраст 31,212,3 года. Данные о проведенном генетическом исследовании имеются лишь у 61 (3,2%) человека, наличие мутации в гене MEN1 подтверждено у 2,9% (55/1914), мутация в гене CDC73 у 0,3% (6/1914) (синдром HPT-JT). Рак ОЩЖ подтвержден у 1,8% (35/1914) пациентов. Хирургическое лечение проведено 64,5% (1234/1914) пациентам. Ремиссия достигнута в 94% (1160/1234) случаев. В 6% случаев зарегистрированы данные о рецидиве или персистенции заболевания после хирургического лечения. Заключение. Отмечается рост выявляемости ПГПТ в РФ по сравнению с 2016 г., что связано с активным началом работы по регистрации пациентов в регионах. На данном этапе требуется продолжение совместной доработки принципов регистрации и мониторинга, что в итоге позволит наладить систему получения информации для расчета необходимых объемов медицинской помощи данной группе.