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Hepatolenticular degeneration

open access: yesБюллетень сибирской медицины, 2005
Hepatolenticular degeneration or Wilson's disease is the hereditary disease, caused by derangement of copper metabolism and its precipitation in brain and internal.
O. Yu. Koryagina   +2 more
doaj   +5 more sources

Hepatolenticular degeneration: diagnostic difficulties (practical experience) [PDF]

open access: yesActa Biomedica Scientifica, 2016
The article deals with severe hereditary disease - hepatolenticular degeneration (Wilson - Konovalov disease). Hepatolenticular degeneration is a monogenic autosomal recessive genetic disorder.
A. V. Ovchinnikov, V. V. Shprakh
doaj   +3 more sources

Wilson’s disease: Food therapy out of trace elements [PDF]

open access: yesFrontiers in Cell and Developmental Biology, 2022
Hepatolenticular degeneration, also known as Wilson’s disease (WD), is an autosomal recessive inheritance nervous disorder of copper metabolism. The treatment of hepatolenticular degeneration emphasizes the combination of medical therapy and dietary ...
Wen-Jie Li   +6 more
doaj   +2 more sources

Clinical observation and risk assessment after splenectomy in hepatolenticular degeneration patients associated with hypersplenism [PDF]

open access: yesFrontiers in Surgery, 2022
BackgroundBoth hepatolenticular degeneration (HLD) and viral hepatitis B (HBV) can cause hypersplenism, but whether splenectomy is needed or can be performed in HLD patients associated with hypersplenism is still controversial.
Wanzong Zhang   +9 more
doaj   +2 more sources

Differentially expressed lncRNAs in liver tissues of TX mice with hepatolenticular degeneration [PDF]

open access: yesScientific Reports, 2021
Wilson's Disease (WD), an ATP7B-mutated inherited disease that affects copper transport, is characterised by liver and nervous system manifestations. Long non-coding (ln-c) RNAs are widely involved in almost all physiological and pathological processes ...
Juan Zhang   +8 more
doaj   +2 more sources

Hepatolenticular degeneration-induced hepatic dysfunction with extremely atypical clinical manifestations: a Case Report [PDF]

open access: yesFrontiers in Medicine
Hepatolenticular Degeneration (HLD) is a rare condition caused by a genetic copper metabolism disorder and a basal ganglia-dominated degenerative brain disease. Its characteristic clinical features include progressive extrapyramidal symptoms, psychiatric
Zhuang Tao   +5 more
doaj   +2 more sources

Clinical case of fulminant hepatitis in a child with hepatolenticular degeneration

open access: yesZdorovʹe Rebenka, 2021
The article presents a detailed analysis of the diagnostic search for Wilson’s disease in childhood. Polymorphism of clinical symptoms of the disease has led to various descriptions of this pathology and its manifestations. Despite more than a century of
I.M. Nesina   +4 more
doaj   +1 more source

Prevalence of hepatolenticular degeneration in different countries

open access: yesKlinicist, 2023
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 ...
E. V. Ovchinnikova   +3 more
doaj   +1 more source

A rare giant intracranial arachnoid cyst confused the diagnosis and treatment of Wilson disease

open access: yesTranslational Neuroscience, 2022
Hepatolenticular degeneration (HLD), also known as Wilson disease (WD), is a rare autosomal-recessive hereditary disease, which is often missed and misdiagnosed because of its various clinical manifestations.
Wenbin Zhang   +4 more
doaj   +1 more source

Inhibition of ASIC1a-Mediated ERS Improves the Activation of HSCs and Copper Transport Under Copper Load

open access: yesFrontiers in Pharmacology, 2021
Hepatolenticular degeneration (HLD) is an autosomal recessive genetic disease caused by the toxic accumulation of copper in the liver. Excessive copper will disrupt the redox balance in cells and tissues, causing ischemia, hypoxia, and inflammation. Acid-
Lingjin Kong   +5 more
doaj   +1 more source

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