Prevalence of hepatolenticular degeneration in different countries

Introduction. Despite modern advances in diagnostic technologies, the problem of Wilson (WD) disease prevalence remains relevant in the world; clinical and scientific interest in epidemiological studies varies in the Russian Federation and in different countries.The aim. To study the prevalence of W...

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Main Authors: E. V. Ovchinnikova, N. A. Schneider, A. A. Ovchinnikova, R. F. Nasyrova
Format: Article
Language:Russian
Published: ABV-press 2023-10-01
Series:Klinicist
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Online Access:https://klinitsist.abvpress.ru/Klin/article/view/547
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author E. V. Ovchinnikova
N. A. Schneider
A. A. Ovchinnikova
R. F. Nasyrova
author_facet E. V. Ovchinnikova
N. A. Schneider
A. A. Ovchinnikova
R. F. Nasyrova
author_sort E. V. Ovchinnikova
collection DOAJ
description Introduction. Despite modern advances in diagnostic technologies, the problem of Wilson (WD) disease prevalence remains relevant in the world; clinical and scientific interest in epidemiological studies varies in the Russian Federation and in different countries.The aim. To study the prevalence of WD in the population of the Russian Federation and foreign countries at the present stage.Materials and methods. The paper presents an analysis of the results of domestic and foreign epidemiological studies of WD published in the eLibrary, Google Scholar, PubMed, Springer, Scopus, Elsevier, Oxford Press, Clinical Case, Cochrane, Science Direct databases from January 01, 2018 to June 01, 2023 year.Results. In total, 14 epidemiological studies were analyzed. In the countries of North and South America, the prevalence of WD is from 4.1 per 100,000, up to 6.4 per 100,000 and in European countries from 1.44 per 100,000 to 9.0 per 100,000, in Asian countries – from 1.793 per 100,000 to 4.81 per 100,000.Conclusion. Results of epidemiological studies prevalence of WD depended on design and methodology: with using the method of genetic screening of the population for WD, the prevalence rates were much higher and reached 40 per 100,000 in Europe, 19.6 in Asia due to detection of early stages of disease development (asymptomatic forms).
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spelling doaj-art-6423e07e05be4d2f8605c81b27d4b6122025-08-20T04:00:43ZrusABV-pressKlinicist1818-83382023-10-01172121810.17650/1818-8338-2023-17-2-K681404Prevalence of hepatolenticular degeneration in different countriesE. V. Ovchinnikova0N. A. Schneider1A. A. Ovchinnikova2R. F. Nasyrova3Far Eastern Federal University, Ministry of Science and Higher Education of Russia; V.M. Bekhterev National Medical Research Center of Psychiatry and Neurology, Ministry of Health of Russia; Primorsky Regional Clinical Hospital No. 1, Ministry of Health of RussiaV.M. Bekhterev National Medical Research Center of Psychiatry and Neurology, Ministry of Health of Russia; prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of RussiaFar Eastern Federal University, Ministry of Science and Higher Education of RussiaV.M. Bekhterev National Medical Research Center of Psychiatry and Neurology, Ministry of Health of RussiaIntroduction. Despite modern advances in diagnostic technologies, the problem of Wilson (WD) disease prevalence remains relevant in the world; clinical and scientific interest in epidemiological studies varies in the Russian Federation and in different countries.The aim. To study the prevalence of WD in the population of the Russian Federation and foreign countries at the present stage.Materials and methods. The paper presents an analysis of the results of domestic and foreign epidemiological studies of WD published in the eLibrary, Google Scholar, PubMed, Springer, Scopus, Elsevier, Oxford Press, Clinical Case, Cochrane, Science Direct databases from January 01, 2018 to June 01, 2023 year.Results. In total, 14 epidemiological studies were analyzed. In the countries of North and South America, the prevalence of WD is from 4.1 per 100,000, up to 6.4 per 100,000 and in European countries from 1.44 per 100,000 to 9.0 per 100,000, in Asian countries – from 1.793 per 100,000 to 4.81 per 100,000.Conclusion. Results of epidemiological studies prevalence of WD depended on design and methodology: with using the method of genetic screening of the population for WD, the prevalence rates were much higher and reached 40 per 100,000 in Europe, 19.6 in Asia due to detection of early stages of disease development (asymptomatic forms).https://klinitsist.abvpress.ru/Klin/article/view/547hepatolenticular degenerationwilson–konovalov diseasecopper metabolismcasual mutation in the atp7b geneasymptomatic form of the diseaseneurological disordersextrapyramidal manifestationsorphan diseaseprevalencefrequency of occurrenceepidemiology
spellingShingle E. V. Ovchinnikova
N. A. Schneider
A. A. Ovchinnikova
R. F. Nasyrova
Prevalence of hepatolenticular degeneration in different countries
Klinicist
hepatolenticular degeneration
wilson–konovalov disease
copper metabolism
casual mutation in the atp7b gene
asymptomatic form of the disease
neurological disorders
extrapyramidal manifestations
orphan disease
prevalence
frequency of occurrence
epidemiology
title Prevalence of hepatolenticular degeneration in different countries
title_full Prevalence of hepatolenticular degeneration in different countries
title_fullStr Prevalence of hepatolenticular degeneration in different countries
title_full_unstemmed Prevalence of hepatolenticular degeneration in different countries
title_short Prevalence of hepatolenticular degeneration in different countries
title_sort prevalence of hepatolenticular degeneration in different countries
topic hepatolenticular degeneration
wilson–konovalov disease
copper metabolism
casual mutation in the atp7b gene
asymptomatic form of the disease
neurological disorders
extrapyramidal manifestations
orphan disease
prevalence
frequency of occurrence
epidemiology
url https://klinitsist.abvpress.ru/Klin/article/view/547
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AT rfnasyrova prevalenceofhepatolenticulardegenerationindifferentcountries