Results 31 to 40 of about 177,432 (192)

Acute-Onset Optic Neuropathy in Wilson’s Disease

Case Reports in Ophthalmology, 2019
Wilson’s disease (WD), also known as hepatolenticular degeneration, is a rare autosomal recessive condition of excess copper accumulation that is most commonly associated with hepatic, neurologic, psychiatric, and ocular manifestations.
Liyung Tiffany Chou, Derek Horkey, Mark Slabaugh   +2 more
doaj   +2 more sources

Metabolic balances of sulfur in patients with hepatolenticular degeneration and effect of the use of D-penicillamine Balanços metabólicos do enxôfre em pacientes com degeneração hepatolenticular e efeito da administração de D-penicilamina

Arquivos de Neuro-Psiquiatria, 1969
The metabolic balances of sulfur in two cases of hepatolenticular degeneration were studied. A positive balance was found in both cases; in one patient submitted to mixed diet, the average was significantly higher than the normal mean. The administration
Horacio M. Canelas, Francisco B. De Jorge, Waldir A. Tognola   +2 more
doaj   +1 more source

IgG4-related autoimmune hepatitis: A case report

Journal of International Medical Research, 2023
IgG-4-related autoimmune hepatitis (IgG4-AIH) is a rare disease. We report here a case of IgG4-AIH in an elderly male patient who was admitted to hospital because of unexplained hepatic insufficiency.
Yunpeng Li, Lifeng Wang
doaj   +2 more sources

Sleep disorders in hepatolenticular degeneration: a primary study

Chinese Journal of Contemporary Neurology and Neurosurgery, 2016
Objective To investigate the characteristics and potential mechanisms of sleep disorders in patients with hepatolenticular degeneration (HLD), also known as Wilson's disease (WD).
Zhi-fei YOU, Xiao-ping WANG
doaj   +1 more source

Copper and ceruloplasmin contents in the blood serum of peripheral and pre-hepatic veins Concentrações de cobre e ceruloplasmina no soro sanguíneo de veias periférica e pré-hepática

Arquivos de Neuro-Psiquiatria, 1976
Copper and ceruloplasmin contents were determined in samples of peripheral and pre-hepatic venous blood of 11 patients with Manson's schistosomiasis and one patient with hepatolenticular degeneration, all of çhich submitted either to porto-caval or ...
H. M. Canelas, I. Roitman, M. Scaff, S. Raia, F. B. De Jorge   +4 more
doaj   +1 more source

Chinese Multidisciplinary Expert Consensus on Orphan/Anticopper Drugs and Other Non-drug Management of Hepatolenticular Degeneration [PDF]

Current Neuropharmacology
Qiang Xia, Xiaoping Wang, Ren-Min Yang
exaly   +2 more sources

Wilson’s disease: Food therapy out of trace elements

Frontiers 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, Wen-Jie Li, Huan-Ling Chen, Bin Wang, Lei Yao, Xiao-Ping Wang, Xiao-Ping Wang   +6 more
doaj   +1 more source

Glymphatic System Dysfunction and its Associated Factors in Wilson's Disease: A DTI-ALPS Study. [PDF]

Brain Behav
Glymphatic function, assessed by DTI‐ALPS index, was impaired in neurological Wilson's disease compared with hepatic phenotype and controls. Age, AST, and UWDRS‐I score independently predicted dysfunction, linking neurodegeneration, liver injury, and altered glymphatic activity. ABSTRACT Background Wilson's disease (WD) is a genetic disorder of copper (
Zhu L, Wu T, Zhang LJ, Liu QY, Zhang LL, Song B, Zhu YL, Hua L, Zhang L, Li B, Sun Q, Xue BC, Xu Y, Han YZ, Han YS.   +14 more
europepmc   +2 more sources

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

Translational 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, Yeqing Huang, Aiqun Liu, Mingfan Hong, Zhisheng Wei   +4 more
doaj   +1 more source

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

Frontiers 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, Huiping Huang, Shaohua Luan, Hui Liu, Manping Ye, Fanrong Wu   +5 more
doaj   +1 more source