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Thursday, March 5, 2020

Wilson’s disease: Asymptomatic patients treatment protocol


Wilson’s disease

Asymptomatic patients treatment protocol:

Initial treatment (duration ranges from 6 months to 5 years): 
- Trientine 750– 1500mg/ day in two or three divided doses; or penicillamine 250– 500mg/ day, increasing by 250mg every 4– 7 days to 1000– 1500mg/ day in two divided doses;

- zinc 50mg tds if chelator not tolerated or declined.

- A djunctive treatment: avoid copper- rich foods (shellfish, nuts, chocolate, mushrooms, organ meats), especially during year 1; pyridoxine 25– 50mg daily (penicillamine inactivates pyridoxine).

-Maintenance (lifelong):
• zinc 50mg tds or lower dose (approximately two- thirds initial dose) of chelating agent.

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