What is the prognosis of Wilson disease?

Updated: Feb 14, 2019
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Patients with a prognostic index (ie, score) of 7 or greater should be considered for liver transplantation (see Table 1, below). All patients in the study associated with this prognostic index who exceeded this score died within 2 months of diagnosis, irrespective of the institution of appropriate medical therapy.

Table. Prognostic Index in Fulminant Wilsonian Hepatitis (Open Table in a new window)

Score

0

1

2

3

4

Serum bilirubin (reference range, 3-20 mmol/L)

< 100

100-150

151-200

201-300

>300

Serum aspartate transaminase (reference range, 7-40 IU/L)

< 100

100-150

151-200

201-300

>300

Prothrombin time prolongation (seconds)

< 4

4-8

9-12

13-20

>30

Prognosis after liver transplantation is relatively good. In a study involving 55 patients with Wilson disease who underwent hepatic transplantation, the 1-year survival rate was 79% and the overall survival rate was 72% at 3 months to 20 years. Another study of 32 patients reported a 1-year survival of 90.6%, a 5-year survival rate of 83.7%, and a 10-year survival rate of 79.9% after living donor liver transplantation. [11]

Important clues for the diagnosis of Wilson disease that a clinician must recognize are a younger patient with hemolytic anemia, impaired hepatic synthetic function, and normal alkaline phosphatase values.


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