Table I.

Copper metabolism tests at the time of diagnosis and at the time of study in 32 Wilson’s disease patients.

	All patients (n=32)	Hepatic presentation (n=11)	Neurological presentation (n=21)	p
Duration of treatment (years)	12.1±11.7	11.4±11.1	12.0±11.6	0.44
Baseline ceruloplasmin serum concentration (mg/dl)**	6.4±4.9	5.4±5.0	5.5±4.8	0.72
Baseline total serum copper (μg/l)**	34.2±22.6	38.7±23.2	38.2±20.4	0.52
Baseline NCC (μg/l)***†	18.2±14.2	18.1±15.1	17.1±13.4	0.76
NCC at the time of study (μg/l) ***†	8.0±7.7	6.0±7.8	7.9±6.7	0.42
24-h urinary copper excretion at diagnosis (μg/24 h)**	657.8±582.6	695.0±613.6	644.9±600.5	0.72
24-h urinary copper excretion at the time of study (μg/24 h)**	653.9±354.1	683.3±391.1	679.2±386.1	0.74
24-h urinary copper excretion after DPA interruption (μg/24h)**	48.4±44.6	48.7±48.42	44.68±40.51	0.65
Non-compliant patients*	10 (31.2%)	3(27.2%)	7(33.3%)	0.29

Data are presented as mean±SD or

^*number of observations (%)

^**Normal ranges of copper metabolism tests: serum ceruloplasmin concentration 25.0–45.0 mg/dl, serum copper concentration 70.0–140.0 μg/dl, and urinary copper excretion <50 μg/24 h.

^***according to European and American recommendations normal values for free copper concentration are in the range 10–15 μg/dl. Negative values of NCC: in 5 patients at the time of diagnosis (2 with hepatic features, 3 with neurological features) and in 12 patients at the time of study (5 with hepatic features, 7 with neurological features).

Abbrevations: NCC=non-ceruloplasmin-bound copper; DPA=d-penicillamine.