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Table 1 Characteristics of Wilson disease patients

From: Wilson disease in Northern Portugal: a long-term follow-up study

 

Sex

Age at diagnosis (years old)

Familiar history

Predominant features

Neurologic symptoms

Kayser–Fleischer rings

Staging of liver fibrosis (Metavir score)

Hepatic liver failure

Treatment

Liver transplant

Outcome

Follow-up (years)

1

Male

18

No

Hepatic

No

No

F4

No

Penicillamine

No

Alive

29

2

Male

22

No

Neuropsychiatric

Yes

Yes

F3

No

Penicillamine >> Trientin

No

Alive

4

3

Male

43

No

Hepatic

No

No

F4

No

Trientin

No

Alive

4

4

Female

15

No

Hepatic

No

No

F1

No

Zinc

No

Alive

25

5

Female

12

No

Hepatic

No

No

F2

No

Penicillamine

No

Alive

8

6

Male

30

No

Hepatic

No

Yes

F4

No

Penicillamine

Yes

Alive

20

7

Female

8

No

Hepatic

No

No

F2

No

Trientin

No

Alive

14

8

Female

13

Yes

Neuropsychiatric

Yes

Yes

F4

Yes

Yes

Alive

15

9

Female

16

No

Hepatic

No

No

F4

No

Yes

Alive

29

10

Male

22

Yes

Hepatic

No

Yes

F4

No

Yes

Alive

14

11

Male

18

No

Ophthalmologic

No

Yes

F1

No

Penicillamine

No

Alive

32

12

Male

25

No

Neuropsychiatric

Yes

Yes

F4

No

Yes

Alive

20

13

Female

25

No

Neuropsychiatric

Yes

Yes

F4

No

Penicillamine

No

Death

12

14

Male

8

No

Hepatic

No

No

F1

No

Trientin

No

Alive

18

15

Male

18

Yes

Hepatic

No

No

F4

No

Penicillamine >> Trientin

No

Alive

17

16

Male

9

Yes

Hepatic

No

Yes

F4

No

Penicillamine

No

Alive

18

17

Male

22

No

Hepatic

No

Yes

F4

Yes

Yes

Alive

22

18

Female

50

No

Hepatic

No

No

F2

No

Penicillamine >> Trientin

No

Alive

15

19

Female

18

No

Hepatic

No

Yes

F4

Yes

Yes

Alive

22

20

Male

25

No

Neuropsychiatric

Yes

Yes

F4

No

Yes

Death

7

21

Female

16

No

Neuropsychiatric

Yes

Yes

F4

No

Penicillamine

Yes

Alive

19

22

Female

19

No

Hepatic

Yes

Yes

F4

Yes

Yes

Alive

16

23

Male

21

Yes

Hepatic

Yes

No

F2

No

Trientin

No

Alive

20

24

Female

32

No

Hepatic

No

No

F2

No

Penicillamine >> Trientin

No

Alive

20