[PDF][PDF] The prevalence of Wilson's disease: an update

TD Sandahl, TL Laursen, DE Munk, H Vilstrup… - …, 2020‏ - Wiley Online Library
Background and Aims In 1984, Scheinberg and Sternlieb estimated the prevalence of
Wilson's disease to be 1: 30,000 based on the limited available data. This suggested a large …

Investigation and management of Wilson's disease: a practical guide from the British Association for the Study of the Liver

S Shribman, T Marjot, A Sharif… - The Lancet …, 2022‏ - thelancet.com
Wilson's disease is an autosomal-recessive disorder of copper metabolism with hepatic,
neurological, psychiatric, ophthalmological, haematological, renal, and rheumatological …

Liver disease in women: the influence of gender on epidemiology, natural history, and patient outcomes

J Guy, MG Peters - Gastroenterology & hepatology, 2013‏ - pmc.ncbi.nlm.nih.gov
Women more commonly present with acute liver failure, autoimmune hepatitis, benign liver
lesions, primary biliary cirrhosis, and toxin-mediated hepatotoxicity. Women less commonly …

[PDF][PDF] Age and sex but not ATP7B genotype effectively influence the clinical phenotype of Wilson disease

P Ferenci, W Stremmel, A Członkowska, F Szalay… - …, 2019‏ - Wiley Online Library
Wilson disease (WD) is an inherited disorder of hepatic copper metabolism with
considerable variation in clinical presentations, the most common ones being liver disease …

Wilson's disease: A 2017 update

A Poujois, F Woimant - Clinics and research in hepatology and …, 2018‏ - Elsevier
Wilson's disease (WD) is characterised by a deleterious accumulation of copper in the liver
and brain. It is one of those rare genetic disorders that benefits from effective and lifelong …

Long-term outcomes of patients with Wilson disease in a large Austrian cohort

S Beinhardt, W Leiss, AF Stättermayer… - Clinical …, 2014‏ - Elsevier
Background & Aims Wilson disease is an autosomal recessive disorder that affects copper
metabolism, leading to copper accumulation in liver, central nervous system, and kidneys …

Wilson's disease: overview

A Lucena-Valera, P Ruz-Zafra, J Ampuero - Medicina Clínica (English …, 2023‏ - Elsevier
Wilson's disease (WD) is an uncommon hereditary disorder caused by a deficiency in the
ATP7B transporter. The protein codified by this gene facilitates the incorporation of the …

Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease

A Poujois, R Sobesky, WG Meissner, AS Brunet… - Neurology, 2020‏ - neurology.org
Objective To evaluate the effect of liver transplantation (LT) in patients with Wilson disease
(WD) with severe neurologic worsening resistant to active chelation. Methods French …

Early neurological worsening in patients with Wilson's disease

T Litwin, K Dzieżyc, M Karliński, G Chabik… - Journal of the …, 2015‏ - Elsevier
Background Early neurological worsening during treatment initiation for Wilson's disease
(WD) is an unresolved problem. Our aim was to establish the frequency and outcome of …

Copper and the brain noradrenergic system

S Lutsenko, C Washington-Hughes, M Ralle… - JBIC Journal of …, 2019‏ - Springer
Copper (Cu) plays an essential role in the development and function of the brain. In humans,
genetic disorders of Cu metabolism may cause either severe Cu deficiency (Menkes …