From: Comparative assessment of clinical rating scales in Wilson’s disease
N | Percentage | Median | Range | |
---|---|---|---|---|
Gender | ||||
Female | 33 | 50.8 | ||
Male | 32 | 49.2 | ||
Age at assessment (yr) | ||||
All | 35 | 15–62 | ||
Female | 40 | 15–62 | ||
Male | 29.5 | 16–62 | ||
Age at onset of symptoms (yr) | 16 | 1–47 | ||
Age at time of diagnosis (yr) | 17 | 3–54 | ||
Initial mode of manifestation | ||||
Hepatic | 46 | 70.8 | ||
Hepatic + neurological | 10 | 15.4 | ||
Neurological | 6 | 9.2 | ||
Asymptomatic | 3 | 4.6 | ||
Liver cirrhosis at time of diagnosis | 21 | 32.3 | ||
By initial mode of manifestation | ||||
Hepatic [n = 46] | 15 | 32.6 | ||
Hepatic + neurological [n = 10] | 6 | 60.0 | ||
Liver status at assessment | ||||
MELD score [missing data n = 4] | 7.3 | 6–17 | ||
Child-Pugh score [missing data n = 7] | 5 | 5–11 | ||
KFR at time of diagnosis [missing data n = 8] | 28 | 49.1 | ||
KFR at assessment | 19 | 29.2 | ||
Treatment at assessment in all patients (n = 65) | ||||
D-Penicillamine | 38 | 58.5 | ||
Trientine | 16 | 24.6 | ||
Zinc | 7 | 10.8 | ||
D-Penicillamine + zinc | 2 | 3.1 | ||
Trientine + zinc | 2 | 3.1 | ||
Treatment at assessment in neurologically symptomatic patients (n = 16) | ||||
Chelating agents | 13 | 81.2 | ||
Zinc with or without chelating agent | 3 | 18.8 | ||
Duration of treatment (yr) | 15 | 0.4–47 |