•Outcome in
wilson disease
•
•Despite
adequate chelation therapy the outcome is unpredictable with 48% mortality in
hospital series. Common outcome seen are:
–Rapid
and complete improvement of hepatic lesions including early cirrhosis
–Initial
deterioration of neurological symptoms but with eventual improvement with few
residual handicaps (speech and handwriting)
–Relentless
progression and death as in fulminant hepatic failure
–Relentless
progression and death in advanced cirrhosis
–Normal
healthy outcome in asymptomatic siblings of index patient who take regular
chelation therapy