ketendoКлиническая и экспериментальная тиреоидологияClinical and experimental thyroidology1995-54722310-3787Endocrinology Research Centre10.14341/ket12802ketendo-12802Research ArticleОригинальные исследованияOriginal StudiesКлинический портрет пациентов с хроническим гипопаратиреозом по данным Всероссийского регистраClinical profile of patients with chronic hypoparathyroidism according to the All-Russian registryhttps://orcid.org/0000-0001-8694-9679СалимхановР. Х.SalimkhanovR. Kh.

Салимханов Рустам Халилович

117036, Москва, ул. Дм. Ульянова, д. 11

Rustam Kh. Salimkhanov, MD

11 Dm. Ulyanova street, 117036 Moscow

rustam.salimkhanov@gmail.comhttps://orcid.org/0000-0002-9258-2591КовалеваЕ. В.KovalevaE. V.

Ковалева Елена Владимировна, к.м.н.

Москва

Elena V. Kovaleva, MD, PhD

Moscow

elen.v.kovaleva@gmail.comhttps://orcid.org/0000-0001-6935-3187ЕлфимоваА. Р.ElfimovaA. R.

Елфимова Алина Ринатовна

Москва

Alina R. Elfimova

Moscow

9803005@mail.ruhttps://orcid.org/0000-0001-6667-062XЕремкинаА. К.EremkinaA. K.

Еремкина Анна Константиновна, к.м.н.

Москва

Anna K. Eremkina, MD, PhD

Moscow

a.lipatenkova@gmail.comhttps://orcid.org/0000-0002-9462-8522Першина-МилютинаА. П.Pershina-MiliutinaA. P.

Першина-Милютина Анастасия Павловна

Москва

Anastasia P. Pershina-Miliutina

Moscow

oa11111998@gmail.comhttps://orcid.org/0000-0001-5952-5846БибикЕ. Е.BibikE. E.

Бибик Екатерина Евгеньевна, к.м.н. 

Москва

Ekaterina E. Bibik, MD, PhD

Moscow

bibikaterina@mail.ruhttps://orcid.org/0000-0003-2669-9457ГорбачеваА. М.GorbachevaA. M.

Горбачева Анна Максимовна, к.м.н.

Москва

Anna M. Gorbacheva, MD, PhD

Moscow

ann.gorbachewa@yandex.ruhttps://orcid.org/0000-0003-0571-8882ВикуловаО. К.VikulovaO. K.

Викулова Ольга Константиновна, д.м.н., доцент

Москва

Olga K. Vikulova, MD, PhD, associate professor

Moscow

gos.registr@endocrincentr.ruhttps://orcid.org/0000-0002-9717-9742МокрышеваН. Г.MokryshevaN. G.

Мокрышева Наталья Георгиевна, д.м.н., профессор, член-корр. РАН

Москва

Natalia G. Mokrysheva, MD, PhD, Professor, corresponding member of the Russian Academy of Sciences

Moscow

mokrisheva.natalia@endocrincentr.ruНациональный медицинский исследовательский центр эндокринологииРоссияEndocrinology Research CentreRussian Federation2024121120242022737Copyright © Салимханов Р.Х., Ковалева Е.В., Елфимова А.Р., Еремкина А.К., Першина-Милютина А.П., Бибик Е.Е., Горбачева А.М., Викулова О.К., Мокрышева Н.Г., 20242024Салимханов Р.Х., Ковалева Е.В., Елфимова А.Р., Еремкина А.К., Першина-Милютина А.П., Бибик Е.Е., Горбачева А.М., Викулова О.К., Мокрышева Н.Г.Salimkhanov R.K., Kovaleva E.V., Elfimova A.R., Eremkina A.K., Pershina-Miliutina A.P., Bibik E.E., Gorbacheva A.M., Vikulova O.K., Mokrysheva N.G.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.cet-endojournals.ru/jour/article/view/12802ОБОСНОВАНИЕ

ОБОСНОВАНИЕ. Хронический гипопаратиреоз (ГипоПТ) — редкое эндокринное заболевание, требующее пожизненной многокомпонентной терапии. Целью лечения ГипоПТ является достижение целевых значений основных показателей кальций-фосфорного обмена, прежде всего — кальциемии, а также профилактика острых и отсроченных осложнений, включая патологию почек, органа зрения, головного мозга, других тканей и систем. Одним из способов повышения качества медицинской помощи, определения оптимальных клинических и терапевтических стратегий ведения пациентов, а также поиска прогностических маркеров ГипоПТ является анализ крупных баз данных. Указанный подход позволяет не только лучше понять особенности течения заболевания, но и оценить эффективность различных схем терапии.

ЦЕЛЬ

ЦЕЛЬ. Оценить клинико-биохимический профиль, медикаментозную терапию и долгосрочные осложнения у пациентов с хроническим послеоперационным (п/о) и нехирургическим (н/х) ГипоПТ по данным Всероссийского регистра.

МАТЕРИАЛЫ И МЕТОДЫ

МАТЕРИАЛЫ И МЕТОДЫ. На базе Всероссийского регистра пациентов с хроническим п/о и н/х ГипоПТ проведено обсервационное, непрерывное исследование. В исследование включены 1776 пациентов из 81 субъекта Российской Федерации (РФ).

РЕЗУЛЬТАТЫ

РЕЗУЛЬТАТЫ. В исследуемой популяции хронический ГипоПТ преобладал среди женщин (86,5%), большинство пациентов имели п/о этиологию заболевания (70,1%), при этом наиболее часто хронический п/о ГипоПТ развивался вследствие хирургических вмешательств на органах шеи по поводу высокодифференцированного рака щитовидной железы (ЩЖ) (44,1%). Целевые значения кальциемии были достигнуты у 44,6% пациентов, фосфатемии — у 54,7%. При анализе ассоциированных осложнений структурная патология почек (нефрокальциноз/нефролитиаз) наблюдались у 33,4% и 10,7%, соответственно; снижение рСКФ до хронической болезни почек (ХБП) 3а-5 стадий — у 17,4%. Катаракта диагностирована в 34,7% случаев. В исследуемой популяции минеральная плотность костной ткани (МПК) в основных отделах (поясничный отдел позвоночника, бедренная и лучевая кости) находилась в пределах ожидаемых по возрасту значений вне зависимости от этиологии ГипоПТ, признаков феномена высокой костной плотности на наблюдалось. Трабекулярный костный индекс (ТКИ) соответствовал нормальной костной микроархитектонике. 70,4% пациентов получали классическую терапию ГипоПТ — комбинацию препаратов активных метаболитов витамина D и кальция. Дополнительные препараты (магния, рекомбинантного человеческого ПТГ (рчПТГ), тиазидные диуретики) назначались в 5,9% случаев.

ЗАКЛЮЧЕНИЕ

ЗАКЛЮЧЕНИЕ. Эпидемиологические сведения о распространенности и заболеваемости ГипоПТ в РФ ограничены, что прежде всего связано с отсутствием официального статистического учета данной нозологии. Изучение анамнестических, лабораторно-инструментальных характеристик ГипоПТ у пациентов в российской популяции — важный этап на пути оптимизации лечения и диагностики заболевания в данной когорте пациентов. Проведенный анализ указывает на недостаточный лабораторный контроль за заболеванием, а также охват пациентов в отношении скрининга долгосрочных осложнений. Решение указанной проблемы возможно путем совершенствования действующих клинических рекомендаций, а также повышения осведомленности врачей и пациентов.

BACKGROUND

BACKGROUND. Chronic hypoparathyroidism (HypoPT) is a rare endocrine disorder that requires lifelong multi-component therapy. The goal of HypoPT treatment is to reach the target values of the main indicators of calcium-phosphorus metab olism, first of all — calciemia, as well as to prevent acute and delayed complications, including pathology of kidneys, eyes, brain and other organs. One of the ways to improve the quality of medical care, determine optimal clinical and therapeutic management strategies, and find prognostic markers for HypoPT is to analyze large databases. This approach allows not only a better understanding of the peculiarities of disease progression, but also the evaluation of the efficacy of different therapeutic regimens.

AIM

AIM. To evaluate the clinical and biochemical profile, medication therapy, and long-term complications in patients with chronic postoperative and nonsurgical HypoPT according to the data of the Russian Registry.

MATERIALS AND METHODS

MATERIALS AND METHODS. The observational, continuous study was based on the data of the Russian Registry of Patients with Chronic Postoperative and Nonsurgical HypoPT; 1776 patients from 81 regions of the Russian Federation were included in the study. RESULTS. In the study population, chronic HypoPT was predominant in women (86.5%), most patients had a postoperative etiology of the disease (70.1%), with the most common development of chronic postoperative HypoPT due to neck surgery for highly differentiated thyroid cancer (44.1%). Target calciemia was achieved in 44.6% of patients and target phosphatemia in 54.7%. Structural renal pathology (nephrocalcinosis/nephrolithiasis) was observed in 33.4% and 10.7% of patients, re spectively, and a decrease in glomerular filtration rate to chronic kidney disease stages 3a-5 in 17.4% of patients. Cataract occurred in 34.7%. In general, bone mineral density in the main zones (lumbar spine, femur, and radius) was within the values expected for the age of patients with both postoperative and nonsurgical HypoPT, and there was no evidence of high bone density phenomenon. The trabecular bone index corresponded to normal bone microarchitecture. 70.4% of patients re ceived classical HypoPT therapy — a combination of preparations of active metabolites of vitamin D and calcium. Additional medications (magnesium, potassium, recombinant human PTH, thiazide diuretics) were present in 5.9% of patients.

CONCLUSION

CONCLUSION. Currently, there are limited epidemiologic data on the prevalence and morbidity of HypoPT in the Russian Federation, mainly due to the lack of nosology in the official statistical forms. The study of anamnestic, laboratory and in strumental characteristics of HypoPT in patients of the Russian population is an important step on the way to optimize the treatment and diagnosis of the disease. The analysis shows that the laboratory control of the disease is inadequate, as well as the coverage of patients with regard to the screening for long-term complications. Improving current clinical guidelines and raising awareness among physicians and patients can help overcome this problem.

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The Russian Registry of Chronic Hypoparathyroidism. Front Endocrinol (Lausanne) (2022) 13: doi: https://doi.org/10.3389/FENDO.2022.800119Kovaleva E V., Eremkina AK, Elfimova AR, Krupinova JA, Bibik EE, Maganeva IS, Gorbacheva AM, Dobreva EA, Melnichenko GA, Mokrysheva NG. The Russian Registry of Chronic Hypoparathyroidism. Front Endocrinol (Lausanne) (2022) 13: doi: https://doi.org/10.3389/FENDO.2022.800119Clarke BL. Bone disease in hypoparathyroidism. Arq Bras Endocrinol Metabol (2014) 58:545–552. doi: https://doi.org/10.1590/0004-2730000003399Clarke BL. Bone disease in hypoparathyroidism. Arq Bras Endocrinol Metabol (2014) 58:545–552. doi: https://doi.org/10.1590/0004-2730000003399Sakane EN, Vieira MCC, Lazaretti-Castro M, Maeda SS. Predictors of Poor Bone Microarchitecture Assessed by Trabecular Bone Score in Postsurgical Hypoparathyroidism. J Clin Endocrinol Metab (2019) 104:5795–5803. doi: https://doi.org/10.1210/jc.2019-00698Sakane EN, Vieira MCC, Lazaretti-Castro M, Maeda SS. Predictors of Poor Bone Microarchitecture Assessed by Trabecular Bone Score in Postsurgical Hypoparathyroidism. J Clin Endocrinol Metab (2019) 104:5795–5803. doi: https://doi.org/10.1210/jc.2019-00698Tecilazich F, Formenti AM, Frara S, Giubbini R, Giustina A. Treatment of hypoparathyroidism. Best Pract Res Clin Endocrinol Metab (2018) 32:955–964. doi: https://doi.org/10.1016/j.beem.2018.12.002Tecilazich F, Formenti AM, Frara S, Giubbini R, Giustina A. Treatment of hypoparathyroidism. Best Pract Res Clin Endocrinol Metab (2018) 32:955–964. doi: https://doi.org/10.1016/j.beem.2018.12.002Brandi ML, Bilezikian JP, Shoback D, Bouillon R, Clarke BL, Thakker R V., Khan AA, Potts JT. Management of hypoparathyroidism: Summary statement and guidelines. Journal of Clinical Endocrinology and Metabolism (2016) 101:2273–2283. doi: https://doi.org/10.1210/jc.2015-3907Brandi ML, Bilezikian JP, Shoback D, Bouillon R, Clarke BL, Thakker R V., Khan AA, Potts JT. Management of hypoparathyroidism: Summary statement and guidelines. Journal of Clinical Endocrinology and Metabolism (2016) 101:2273–2283. doi: https://doi.org/10.1210/jc.2015-3907Chang YK, Lang BHH. To identify or not to identify parathyroid glands during total thyroidectomy. Gland Surg (2017) 6:S20–S29. doi: https://doi.org/10.21037/gs.2017.06.13Chang YK, Lang BHH. To identify or not to identify parathyroid glands during total thyroidectomy. Gland Surg (2017) 6:S20–S29. doi: https://doi.org/10.21037/gs.2017.06.13

The authors declare that there are no conflicts of interest present.