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
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Hepatolenticular degeneration: diagnostic difficulties (practical experience) [PDF]
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
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Wilson’s disease: Food therapy out of trace elements [PDF]
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
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Clinical observation and risk assessment after splenectomy in hepatolenticular degeneration patients associated with hypersplenism [PDF]
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
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Differentially expressed lncRNAs in liver tissues of TX mice with hepatolenticular degeneration [PDF]
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
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Hepatolenticular degeneration-induced hepatic dysfunction with extremely atypical clinical manifestations: a Case Report [PDF]
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
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Clinical case of fulminant hepatitis in a child with hepatolenticular degeneration
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
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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 ...
E. V. Ovchinnikova +3 more
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A rare giant intracranial arachnoid cyst confused the diagnosis and treatment of Wilson disease
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
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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
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