TABLE 1.
Gender (n,%) | ||
Male | 36 (58.06%) | |
Female | 26 (41.94%) | |
Age at study enrollment (median, IQR) | 41 (30–56) | |
Age at diagnosis of WD (median, IQR) | 19 (11–25) | |
Treatment duration (median, IQR) | 19.5 (7–35) | |
Major depressive disorder (n) (MINI-7) | 22 (37.29%) | |
PHQ 9 score (median, IQR) | 3 (1–6) | |
Cognitive impairment (n) (MOCA <26) | 28 (47.46%) | |
Neurological symptoms (n) (UWDRS >0) | 50 (80.65%) | |
Cirrhosis (n)* | 12 (19.67%) | |
Current WD medication (n) | WD treatment duration median (IQR) | Previous different WD medication regimen (n) |
---|---|---|
d-Penicillamine n = 8 | 37 (4,41) | Y = 0, N = 8 |
Trientine n = 20 | 23.5 (8,43) | Y = 14, N = 6 |
Zinc n = 27 | 17 (12,30) | Y = 25, N = 10 |
Investigational n = 1 | 3 (3,3) | Y = 0, N = 1 |
Chelator and zinc† = 5 | 26 (14,29) | Y = 5, N = 1 |
IQR = interquartile range; MINI = Mini-International Neuropsychiatric Interview; MOCA = Montreal Cognitive Assessment; PHQ = Patient Health Questionnaire; UWDRS = United Wilson Disease Rating Scale; WD = Wilson disease.
See appendix for cirrhosis diagnostic algorithm.
Chelator = d-Penicillamine or Trientine