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 |