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{{Template:Wilson's disease}}
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{{CMG}}
{{CMG}}; {{AE}} {{AEL}}
==Overview==
==Overview==
Medical therapy is the mainstay of therapy for treatment patients with Wilson's disease. The treatment option include copper chelators as penicillamine trientine. The treatment also includes Zinc to prevent copper reaccumulation.


==Medical therapy==
== Recommendations for the treatment of Wilson's Disease (DO NOT EDIT)<ref name="urlwww.aasld.org">{{cite web |url=https://www.aasld.org/sites/default/files/guideline_documents/Wilson%20Disease2009.pdf |title=www.aasld.org |format= |work= |accessdate=}}</ref> ==
Various 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. In general, a diet low in copper-containing foods ([[mushroom]]s, [[nut (fruit)|nuts]],[[chocolate]], dried [[fruit]], liver, and shellfish) is recommended.<ref name=Ala/>
{{cquote|
# Initial treatment for symptomatic patients should include a chelating agent (D-penicillamine or trientine). Trientine may be better tolerated.
# Patients should avoid intake of foods and water with high concentrations of copper, especially during the first year of treatment.
# Treatment of presymptomatic patients or those on maintenance therapy can be accomplished with a chelating agent or with zinc. Trientine may be better tolerated.
 
Treatment in Specific Clinical Situations
 
# Patients with acute liver failure due to WD should be referred for and treated with liver transplantation immediately.
# Patients with decompensated cirrhosis unresponsive to chelation treatment should be evaluated promptly for liver transplantation.
# Treatment for WD should be continued during pregnancy, but dosage reduction is advisable for D-penicillamine and trientine.
# Treatment is lifelong and should not be discontinued, unless a liver transplant has been performed.


Generally, [[penicillamine]] is the first treatment used. This binds copper ([[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% of patients experience a side effect or complicaton 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 also 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=Roberts/> Patients 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=Roberts/> A further agent with known activity in Wilson's disease is [[tetrathiomolybdate]]. This is still regarded as experimental,<ref name=Roberts/>although some studies have shown a beneficial effect.<ref name=Ala/>
Treatment Targets and Monitoring of Treatment


Once all results have returned to normal, [[zinc]] (usually in the form of [[zinc acetate]]) 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 their absorption and transport to the liver. Zinc therapy is continued unless symptoms recur, or if the urinary excretion of copper increases.<ref name=Roberts/>
# For routine monitoring, serum copper and ceruloplasmin, liver biochemistries and international normalized ratio, complete blood count and urinalysis (especially for those on chelation therapy), and physical examination should be performed regularly, at least twice annually. Patients receiving chelation therapy require a complete blood count and urinalysis regularly, no matter how long they have been on treatment.
# The 24-hour urinary excretion of copper while on medication should be measured yearly, or more frequently if there are questions on compliance or if dosage of medications is adjusted. The estimated serum non–ceruloplasmin bound copper may be elevated in situations of non-adherence and extremely low in situations of overtreatment.}}


In rare cases where none of the oral treatments are effective, especially in severe neurological disease, [[dimercaprol]] (British anti-Lewisite) is still occasionally necessary. This treatment is injected [[Intramuscular injection|intermuscularly]] (into a muscle) every few weeks, and has a number of 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=553–5 |year=1996|month=July |pmid=8759497}}</ref>
==Medical therapy==
* The mainstay stay of therapy for treatment of Wilson's disease is the medical therapy. <ref name="Walshe1996">{{cite journal |author=Walshe JM |title=Treatment of Wilson's disease: the historical background |journal=QJM |volume=89 |issue=7 |pages=553–5 |year=1996|month=July |pmid=8759497}}</ref>
* The treatment of Wilson's disease is accomplished through two mechanisms:
** Removing of the excess copper in the body by copper chelators
** Preventing reaccumulation of the copper
* '''Removal of the copper:'''
** Preferred regimen (1):  D-penicillamine 20 mg/kg PO q12h
** Alterantive regimen (1):  Trientine hydrochloride 500 to 750 mg PO q12h/q6h
* '''Preventing reaccumulation:'''
** Preferred regimen (1): Zinc acetate PO 50 mg q8h


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. It is unclear whether these people are best treated with penicillamine or zinc acetate.<ref name=Roberts/>
==References==
==References==


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[[Category:Gastroenterology]]
[[Category:Hematology]]
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Latest revision as of 19:42, 29 August 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

Medical therapy is the mainstay of therapy for treatment patients with Wilson's disease. The treatment option include copper chelators as penicillamine trientine. The treatment also includes Zinc to prevent copper reaccumulation.

Recommendations for the treatment of Wilson's Disease (DO NOT EDIT)[1]

“
  1. Initial treatment for symptomatic patients should include a chelating agent (D-penicillamine or trientine). Trientine may be better tolerated.
  2. Patients should avoid intake of foods and water with high concentrations of copper, especially during the first year of treatment.
  3. Treatment of presymptomatic patients or those on maintenance therapy can be accomplished with a chelating agent or with zinc. Trientine may be better tolerated.

Treatment in Specific Clinical Situations

  1. Patients with acute liver failure due to WD should be referred for and treated with liver transplantation immediately.
  2. Patients with decompensated cirrhosis unresponsive to chelation treatment should be evaluated promptly for liver transplantation.
  3. Treatment for WD should be continued during pregnancy, but dosage reduction is advisable for D-penicillamine and trientine.
  4. Treatment is lifelong and should not be discontinued, unless a liver transplant has been performed.

Treatment Targets and Monitoring of Treatment

  1. For routine monitoring, serum copper and ceruloplasmin, liver biochemistries and international normalized ratio, complete blood count and urinalysis (especially for those on chelation therapy), and physical examination should be performed regularly, at least twice annually. Patients receiving chelation therapy require a complete blood count and urinalysis regularly, no matter how long they have been on treatment.
  2. The 24-hour urinary excretion of copper while on medication should be measured yearly, or more frequently if there are questions on compliance or if dosage of medications is adjusted. The estimated serum non–ceruloplasmin bound copper may be elevated in situations of non-adherence and extremely low in situations of overtreatment.
 ”

Medical therapy

  • The mainstay stay of therapy for treatment of Wilson's disease is the medical therapy. [2]
  • The treatment of Wilson's disease is accomplished through two mechanisms:
    • Removing of the excess copper in the body by copper chelators
    • Preventing reaccumulation of the copper
  • Removal of the copper:
    • Preferred regimen (1): D-penicillamine 20 mg/kg PO q12h
    • Alterantive regimen (1):  Trientine hydrochloride 500 to 750 mg PO q12h/q6h
  • Preventing reaccumulation:
    • Preferred regimen (1): Zinc acetate PO 50 mg q8h

References

  1. ↑ "www.aasld.org" (PDF).
  2. ↑ Walshe JM (1996). "Treatment of Wilson's disease: the historical background". QJM. 89 (7): 553–5. PMID 8759497. Unknown parameter |month= ignored (help)

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