Jump to content
Main menu
Main menu
move to sidebar
hide
Navigation
Main page
Recent changes
Random page
Help about MediaWiki
Special pages
Niidae Wiki
Search
Search
Appearance
Create account
Log in
Personal tools
Create account
Log in
Pages for logged out editors
learn more
Contributions
Talk
Editing
Wilson's disease
(section)
Page
Discussion
English
Read
Edit
View history
Tools
Tools
move to sidebar
hide
Actions
Read
Edit
View history
General
What links here
Related changes
Page information
Appearance
move to sidebar
hide
Warning:
You are not logged in. Your IP address will be publicly visible if you make any edits. If you
log in
or
create an account
, your edits will be attributed to your username, along with other benefits.
Anti-spam check. Do
not
fill this in!
==Treatment== ===Diet=== In general, a diet low in copper-containing foods is recommended. High-copper foods avoided in Wilson's disease include [[mushroom]]s, [[nut (fruit)|nuts]], [[chocolate]], dried [[fruit]], liver, sesame seeds, sesame oil, and [[shellfish]].<ref name=Ala/> ===Medication=== Medical treatments are available for Wilson's disease. Some increase the removal of copper from the body, while others prevent the absorption of copper from the diet. Generally, penicillamine is the first treatment used. This binds to copper (by [[chelation]]) and leads to excretion of copper in the urine. Hence, monitoring of the amount of copper in the urine can be done to ensure a sufficiently high dose is taken. Penicillamine is not without problems; about 20% experience a side effect or complication of penicillamine treatment, such as drug-induced [[systemic lupus erythematosus|lupus]] (causing joint pains and a skin rash) or [[myasthenia gravis|myasthenia]] (a nerve condition leading to muscle weakness). In those who presented with neurological symptoms, almost half experience a paradoxical worsening in their symptoms. While this phenomenon is observed in other treatments for Wilson's, it is usually taken as an indication for discontinuing penicillamine and commencing second-line treatment.<ref name=Ala/><ref name=Roberts2003/> Those intolerant to penicillamine may instead be commenced on [[trientine hydrochloride]], which also has chelating properties. Some recommend trientine as first-line treatment, but experience with penicillamine is more extensive.<ref name=Roberts2003/> A further agent with known activity in Wilson's disease, under clinical investigation by [[Wilson Therapeutics]], is [[bis-choline tetrathiomolybdate|tetrathiomolybdate]]. It is regarded as experimental,<ref name=Roberts2003/> though some studies have shown a beneficial effect.<ref name=Ala/> Once all results have returned to normal, [[zinc]] (usually in the form of a [[zinc acetate]] prescription called Galzin) may be used instead of chelators to maintain stable copper levels in the body. Zinc stimulates [[metallothionein]], a protein in gut cells that binds copper and prevents its absorption and transport to the liver. Zinc therapy is continued unless symptoms recur or if the urinary excretion of copper increases.<ref name=Roberts2003/> In rare cases where none of the oral treatments is effective, especially with severe neurological disease, [[dimercaprol]] (British anti-Lewisite) is occasionally necessary. This treatment is injected [[Intramuscular injection|intramuscularly]] (into a muscle) every few weeks and has unpleasant side effects such as pain.<ref name=Walshe1996>{{cite journal |author=Walshe JM |title=Treatment of Wilson's disease: the historical background |journal=QJM |volume=89 |issue=7 |pages=553β55 |date=July 1996 |pmid=8759497 |doi=10.1093/qjmed/89.7.553|doi-access=free }}</ref> People who are [[asymptomatic]] (for instance, those diagnosed through family screening or only as a result of abnormal test results) are generally treated, as the copper accumulation may cause long-term damage in the future. Whether these people are best treated with penicillamine or zinc acetate is unclear.<ref name=Roberts2003/> ===Physical and occupational therapies=== [[Physiotherapy]] and occupational therapy are beneficial for patients with the neurological form of the disease. The copper-chelating treatment may take up to six months to start working, and these therapies can assist in coping with [[ataxia]], [[dystonia]], and tremors, as well as preventing the development of [[contracture]]s that can result from dystonia.<ref name=Brewer2005>{{cite journal |vauthors=Brewer GJ, Askari FK | title=Wilson's disease: clinical management and therapy | journal=Journal of Hepatology | year=2005 | volume=42 | issue=Suppl 1 | pages=13β21 | pmid=15777568 | doi=10.1016/j.jhep.2004.11.013| doi-access=free }}</ref> ===Transplantation=== [[Liver transplantation]] is an effective cure for Wilson's disease, but is used only in particular scenarios because of the risks and complications associated with the procedure. It is used mainly in people with [[fulminant]] liver failure who fail to respond to medical treatment or in those with advanced chronic liver disease. Liver transplantation is avoided in severe neuropsychiatric illnesses, in which its benefit has not been demonstrated.<ref name=Ala/><ref name=Roberts2003/>
Summary:
Please note that all contributions to Niidae Wiki may be edited, altered, or removed by other contributors. If you do not want your writing to be edited mercilessly, then do not submit it here.
You are also promising us that you wrote this yourself, or copied it from a public domain or similar free resource (see
Encyclopedia:Copyrights
for details).
Do not submit copyrighted work without permission!
Cancel
Editing help
(opens in new window)
Search
Search
Editing
Wilson's disease
(section)
Add topic