Classification and Clinical Heterogeneity of Hepatolenticular Degeneration
https://doi.org/10.52667/2712-9179-2023-3-2-15-24
Abstract
Hepatolenticular degeneration (HLD) or Wilson-Konovalov disease (OMIM277900) is a hereditary monogenic autosomal recessive degenerative disease related to metabolic diseases - a category of storage diseases. HLD has been studied for more than 130 years. During this time, more classifications of this disease were proposed. In this review, we systematized all the proposed classifications of HLD. And we noticed, they are based on the following criteria: 1) clinical signs of the disease; 2) the sequence of their appearance as the pathology progresses (with the primary appearance of signs of liver or brain damage); 3) severity of the disease. This review also systematizes data on the clinical picture of HLD.
About the Authors
E. V. Ovchinnikova
Far Eastern Federal University; V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology; Primorsky Regional Clinical Hospital No. 1
Russian Federation
Russian Federation
Elena V. Ovchinnikova
690922, Vladivostok
192019 St. Petersburg
690091, Vladivostok
E. E. Vaiman
V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology
Russian Federation
Russian Federation
Elena E. Vaiman
192019 St. Petersburg
Tel.: +7-(812)-670-02-20
N. A. Shnayder
V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology; V. F. Voyno-Yasenetsky Krasnoyarsk State Medical University
Russian Federation
Russian Federation
Natalia A. Shnayder
192019 St. Petersburg
660022 Krasnoyarsk
A. A. Ovchinnikova
Far Eastern Federal University
Russian Federation
Russian Federation
Anna A. Ovchinnikova
690922, Vladivostok
R. F. Nasyrova
V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology
Russian Federation
Russian Federation
Regina F. Nasyrova
192019 St. Petersburg
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For citations:
Ovchinnikova E.V., Vaiman E.E., Shnayder N.A., Ovchinnikova A.A., Nasyrova R.F. Classification and Clinical Heterogeneity of Hepatolenticular Degeneration. Personalized Psychiatry and Neurology. 2023;3(2):15-24. https://doi.org/10.52667/2712-9179-2023-3-2-15-24
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