Cerebral Pathology in Hepatolenticular Degeneration (Wilson Disease)
Introduction. Hepatolenticular degeneration (HLD), or Wilson disease, is one of the severe progressive hereditary disorders of the nervous system. A number of questions regarding its pathogenesis and pathology are the subject of in-depth research. The
Tat'yana S. Gulevskaya +2 more
doaj +2 more sources
Treatment of liver fibrosis in hepatolenticular degeneration with traditional Chinese medicine: systematic review of meta-analysis, network pharmacology and molecular dynamics simulation. [PDF]
Background Traditional Chinese medicine (TCM) is widely used in the clinical treatment of hepatolenticular degeneration (HLD) and liver fibrosis (LF). In the present study, the curative effect was assessed using meta-analysis.
Yang X +5 more
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Nomogram-based prediction of portal vein system thrombosis formation after splenectomy in patients with hepatolenticular degeneration. [PDF]
Objective Splenectomy is a vital treatment method for hypersplenism with portal hypertension. However, portal venous system thrombosis (PVST) is a serious problem after splenectomy.
Zheng Z +8 more
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Protective Mechanism of Gandou Decoction in a Copper-Laden Hepatolenticular Degeneration Model: In Vitro Pharmacology and Cell Metabolomics. [PDF]
Gandou decoction (GDD) is a classic prescription for the treatment of hepatolenticular degeneration (HLD) in China; however, the liver-protecting mechanism of this prescription needs further evaluation.
Yin F +8 more
europepmc +2 more sources
[Application of a low copper diet guidance based on food exchange portions in children with hepatolenticular degeneration]. [PDF]
Chen YX, Qiu ZQ, Sun J, Li Y, Yang Y.
europepmc +2 more sources
A novel mutation in the ATP7B gene causing hepatolenticular degeneration in a Chinese family: A case report. [PDF]
Introduction: Hepatolenticular degeneration (Wilson disease) is an autosomal recessive monogenic disorder caused by mutations in the ATPase copper transporting beta (ATP7B) gene located on human chromosome 13.
Zhou Z, Zhang S, Bi Y, Duan W, Gao H.
europepmc +2 more sources
Chinese Multidisciplinary Expert Consensus on Orphan/Anticopper Drugs and Other Non-drug Management of Hepatolenticular Degeneration. [PDF]
Background This study aims to guide the diagnosis and treatment of hepatolenticular degeneration (also named Wilson's disease, WD) and aid multidisciplinary clinicians in making reasonable and personalized treatment regimens.
Yang RM +9 more
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Hepatic Lenticular Degeneration With Renal Tumors: A Case Series. [PDF]
ABSTRACT This article presents a series of cases involving hepatic lenticular degeneration (Wilson's disease) complicated by renal tumors. It details the clinical manifestations, diagnostic procedures, treatment strategies, pathological features and outcomes of these patients. We found that this type of patient has an early‐onset disease, complex tumor
Xiao H +9 more
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A bibliometric analysis of hepatolenticular degeneration research in traditional Chinese medicine using CiteSpace and VOSviewer: A study protocol for systematic review. [PDF]
Background: The purpose of this review was to analyze treatments for hepatolenticular degeneration (HLD) in traditional Chinese medicine (TCM), exploring the potential mechanisms and latest advances, as well as potential future directions.
Tao Z +7 more
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Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy. [PDF]
Long non-coding RNAs (lncRNAs) are a recently discovered group of non-coding RNAs that play a crucial role in the regulation of various human diseases, especially in the study of nervous system diseases which has garnered significant attention.
Hao W +10 more
europepmc +2 more sources

