Primary hyperparathyroidism in Russia according to the registry

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  • Authors: Mokrysheva N.G.1, Mirnaya S.S.2, Dobreva E.A.2, Maganeva I.S.2, Kovaleva E.V.3, Krupinova J.A.2, Kryukova I.V.4, Tevosyan L.K.4, Lukyanov S.V.5, Markina N.V.6, Bondar I.A.7, Podprugina N.G.8, Ignatieva I.A.8, Shabelnikova O.Y.7, Dreval A.V.9, Antsiferov M.B.4, Mel'nichenko G.A.2, Dedov I.I.2
  • Affiliations:
    1. Endocrinology research centre
    2. Endocrinology Research Centre
    3. Endocrinology Research centre
    4. M.F. Vladimirskiy Moscow Regional Research and Clinical Institute
    5. Rostov state medical university
    6. Endocrinological Dispensary of the Moscow Healthcare Department
    7. Novosibirsk State Medical University
    8. Nizhny Novgorod City hospital №33
    9. Moscow Regional Research and Clinical Institute
  • Issue: Vol 65, No 5 (2019)
  • Pages: 300-310
  • Section: Clinical endocrinology
  • URL: https://probl-endojournals.ru/probl/article/view/10126
  • DOI: https://doi.org/10.14341/probl10126
  • Cite item
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Abstract


BACKGROUND: 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.

AIM: Evaluate the frequency of PHPT detection and characterize its clinical forms in Russia using an online registry.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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.


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About the authors

Natalya G. Mokrysheva

Endocrinology research centre

Email: nm70@mail.ru
ORCID iD: 0000-0002-9717-9742
SPIN-code: 5624-3875

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD, PhD, Professor

Svetlana S. Mirnaya

Endocrinology Research Centre

Email: svetlanamirnaya@yahoo.com
ORCID iD: 0000-0002-1341-0397
SPIN-code: 1968-7706

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD, research associate

Ekaterina A. Dobreva

Endocrinology Research Centre

Email: dobrevae@mail.ru
ORCID iD: 0000-0002-8916-7346
SPIN-code: 3405-2467

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD, PhD, senior research associate

Irina S. Maganeva

Endocrinology Research Centre

Author for correspondence.
Email: maganeva.ira@yandex.ru
ORCID iD: 0000-0002-0067-3622
SPIN-code: 2575-3091

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD

Elena V. Kovaleva

Endocrinology Research centre

Email: hypopara.enc@gmail.com
ORCID iD: 0000-0002-9258-2591
SPIN-code: 7387-6791

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

postgraduate student

Julia A. Krupinova

Endocrinology Research Centre

Email: j.krupinova@gmail.com
ORCID iD: 0000-0001-7963-5022
SPIN-code: 6279-8247

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD

Irina V. Kryukova

M.F. Vladimirskiy Moscow Regional Research and Clinical Institute

Email: kiv200877@yandex.ru
ORCID iD: 0000-0002-7876-5105
SPIN-code: 7669-3010

Russian Federation, 61/2, Shchepkina street, Moscow, 129110  

MD, PhD, Assistant Professor

Larisa Kh. Tevosyan

M.F. Vladimirskiy Moscow Regional Research and Clinical Institute

Email: tlh_moscou@mail.ru
ORCID iD: 0000-0002-8954-1633
SPIN-code: 7376-4180

Russian Federation, 61/2, Shchepkina street, Moscow, 129110  

MD

Stanislav V. Lukyanov

Rostov state medical university

Email: svluk@rambler.ru
ORCID iD: 0000-0002-3317-0108
SPIN-code: 4181-8431

Russian Federation, 29, Nachitsevanskij, Rostov-on-Don, 344022

MD, PhD

Natalia V. Markina

Endocrinological Dispensary of the Moscow Healthcare Department

Email: skovran4@gmail.com
ORCID iD: 0000-0003-2784-1726

Russian Federation, 37, Pretchistenka str., Moscow, 119034

MD, PhD

Irina A. Bondar

Novosibirsk State Medical University

Email: bondaria@oblmed.nsk.ru
ORCID iD: 0000-0003-4324-2926
SPIN-code: 6633-8947

Russian Federation, 52, Krasny prospect, Novosibirsk, 630091

MD, PhD, Professor

Natalia G. Podprugina

Nizhny Novgorod City hospital №33

Email: natalia.gec15@mail.ru
ORCID iD: 0000-0003-1538-7335
SPIN-code: 1699-0753

Russian Federation, 54, Lenina pr., Nizhnii Novgorod, 603076

MD, PhD

Irina A. Ignatieva

Nizhny Novgorod City hospital №33

Email: ir.ignatieva-1@yandex.ru
ORCID iD: 0000-0002-2394-240X
SPIN-code: 6187-0270

Russian Federation, 54, Lenina pr., Nizhnii Novgorod, 603076

MD

Olesia Yu. Shabelnikova

Novosibirsk State Medical University

Email: oushab@ngs.ru
ORCID iD: 0000-0003-3906-4784
SPIN-code: 5941-4815

Russian Federation, 52, Krasniy prospect, Novosibirsk, 630091

MD, PhD

Alexander V. Dreval

Moscow Regional Research and Clinical Institute

Email: dreval@diabet.ru
ORCID iD: 0000-0002-3135-9003
SPIN-code: 5853-3989
Scopus Author ID: 6603931817
ResearcherId: F-9632-2017
http://www.researcherid.com/rid/F-9632-2017

Russian Federation, 61/2, Shepkina street, Moscow, 129110

MD, PhD, Professor

Mikhail B. Antsiferov

M.F. Vladimirskiy Moscow Regional Research and Clinical Institute

Email: antsiferov@rambler.ru
ORCID iD: 0000-0002-9944-2997
SPIN-code: 1035-4773

Russian Federation, 61/2, Shepkina street, Moscow, 129110

MD, PhD, Professor

Galina A. Mel'nichenko

Endocrinology Research Centre

Email: teofrast2000@mail.ru
ORCID iD: 0000-0002-5634-7877
SPIN-code: 8615-0038

Russian Federation, 11, Dm. Ulyanova street, Moscow, 117036

MD, PhD, Professor, Academician of the RAS

Ivan I. Dedov

Endocrinology Research Centre

Email: dedov@endocrincentr.ru
ORCID iD: 0000-0002-8175-7886
SPIN-code: 5873-2280

Russian Federation, 11 Dm.Ulyanova street, Moscow, 117036

MD, PhD, Professor

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Supplementary files

Supplementary Files Action
1.
Fig. 1. Change in the classification of PGTT over time.

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2.
Fig. 1. Change in the classification of PGTT over time.

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3.
Fig. 2. Potential number of patients with PGTT in some regions of the Russian Federation.

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4.
Fig. 3. The dynamics of the incidence of PGTT in Moscow according to the Russian Register of Patients with PGTT.

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5.
Fig. 4. Distribution of cases of PGTT in the place of residence of patients in the regions of the Russian Federation *.

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6.
Fig. 5. Prevalence of PGTT in age subgroups, according to the register.

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7.
Fig. 6. Distribution of patients with PGTT by sex and age group, according to the register.

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Copyright (c) 2020 Mokrysheva N.G., Mirnaya S.S., Dobreva E.A., Maganeva I.S., Kovaleva E.V., Krupinova J.A., Kryukova I.V., Tevosyan L.K., Lukyanov S.V., Markina N.V., Bondar I.A., Podprugina N.G., Ignatieva I.A., Shabelnikova O.Y., Dreval A.V., Antsiferov M.B., Mel'nichenko G.A., Dedov I.I.

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