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Treatments for Wilsons disease (hepatolenticular degeneration)  
INFORMATIVE 

Tags: Wilsons disease, autosomal recessive, hereditary disease, heterozygous, neurological, treatments

This disease is treated with lifelong use of chelating agents known as D-penicillamine or trientine hydrochloride, drugs that help remove copper from tissue.

	This disease is treated with lifelong use of chelating agents known as D-penicillamine or trientine hydrochloride, drugs that help remove copper from tissue.
	Patients will also need to take vitamin B6 and follow a low-copper diet, which means avoiding mushrooms, nuts, chocolate, dried fruit, liver, and shellfish.
	Consuming extra zinc may be helpful in blocking the intestines' absorption of copper.
	Liver transplantation is effective in patients with fulminant Wilson's disease that does not respond to the usual treatment.
	Because the primary defect resides within the liver, the transplantation is curative, but as it is only undertaken in severely ill patients the prognosis is still mediocre.

Lister: BobbysWorld
Source: Compiled by LAL Editor

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