Wilson's Disease.
Early diagnosis permits preventive therapy to preempt development of organ damage. In all diagnosed patients, both symptomatic and asymptomatic, pharmacologic therapy is lifelong, and maintenance treatment to prevent copper toxicity is mandatory. Patients with either fulminant hepatic failure or hepatic insufficiency unresponsive to medical therapy should be considered for orthotopic liver transplantation, which effectively cures Wilson's disease. Prognosis is excellent for compliant patients receiving pharmacologic therapy.
Tavill
AS
Mt. Sinai Medical Center at Case Western University, One Mt. Sinai Drive, Cleveland, OH 44106.
Schilsky
ML