Abnormal copper metabolism in cultured fibroblasts from patients with Wilson's disease

1980 ◽  
Vol 3 (1) ◽  
pp. 155-157 ◽  
Author(s):  
J. Camakaris ◽  
L. Ackland ◽  
D. M. Danks
2021 ◽  
pp. 152692482110028
Author(s):  
Alberto Ferrarese ◽  
Patrizia Burra

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.


Hepatology ◽  
1986 ◽  
Vol 6 (3) ◽  
pp. 427-432 ◽  
Author(s):  
Surjit K. S. Srai ◽  
Andrew K. Burroughs ◽  
Bernard Wood ◽  
Owen Epstein

1991 ◽  
Vol 83 (6) ◽  
pp. 364-366 ◽  
Author(s):  
R. Siegemund ◽  
J. Lößner ◽  
K. Günther ◽  
H.-J. Kühn ◽  
H. Bachmann

2006 ◽  
Vol 349 (3) ◽  
pp. 1079-1086 ◽  
Author(s):  
Noriko Fujiwara ◽  
Hiroyuki Iso ◽  
Nobue Kitanaka ◽  
Junichi Kitanaka ◽  
Hironobu Eguchi ◽  
...  

Author(s):  
Vrinda Vijayakumari ◽  
Kaliyannan Mayilananthi ◽  
Durga Krishnan ◽  
Ramprasath Anbazhagan ◽  
Gaurav Narayanan

Wilson’s disease is one the rare autosomal recessive disorders of copper metabolism due to mutation in ATP7B gene located in chromosome 13. The mutations of this gene cause accumulation of copper in different tissues such as brain, liver, and eyes. The clinical presentation usually reflects this tissue distribution and varies from asymptomatic patients to those with hepatic or neuro-psychiatric manifestations. Here, we report an interesting case of Wilson’s disease which presented with mild persistent hemolysis leading to pre hepatic and post hepatic jaundice. He also had hepatocellular jaundice due to liver injury.


1961 ◽  
Vol 40 (3) ◽  
pp. 445-453 ◽  
Author(s):  
James L. German ◽  
Alexander G. Bearn

Molecules ◽  
2021 ◽  
Vol 26 (21) ◽  
pp. 6614
Author(s):  
Pierpaolo Coni ◽  
Giuseppina Pichiri ◽  
Joanna Izabela Lachowicz ◽  
Alberto Ravarino ◽  
Francesca Ledda ◽  
...  

Zinc is the second most abundant trace element in the human body, and it plays a fundamental role in human physiology, being an integral component of hundreds of enzymes and transcription factors. The discovery that zinc atoms may compete with copper for their absorption in the gastrointestinal tract let to introduce zinc in the therapy of Wilson’s disease, a congenital disorder of copper metabolism characterized by a systemic copper storage. Nowadays, zinc salts are considered one of the best therapeutic approach in patients affected by Wilson’s disease. On the basis of the similarities, at histological level, between Wilson’s disease and non-alcoholic liver disease, zinc has been successfully introduced in the therapy of non-alcoholic liver disease, with positive effects both on insulin resistance and oxidative stress. Recently, zinc deficiency has been indicated as a possible factor responsible for the susceptibility of elderly patients to undergo infection by SARS-CoV-2, the coronavirus responsible for the COVID-19 pandemic. Here, we present the data correlating zinc deficiency with the insurgence and progression of Covid-19 with low zinc levels associated with severe disease states. Finally, the relevance of zinc supplementation in aged people at risk for SARS-CoV-2 is underlined, with the aim that the zinc-based drug, classically used in the treatment of copper overload, might be recorded as one of the tools reducing the mortality of COVID-19, particularly in elderly people.


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