Table 1

Patient details: non-fulminant presentation

Patient no	Age at diagnosis (y), sex	Mode of presentation	Caeruloplasmin1-150 (g/l)	Liver copper1-151 (μg/g)	Radiocopper study	Kayser-Fleischer rings	Urine copper1-152 (μg/24 h)	Histology
1	7, F	Hepatic	0.01	1171	–	Absent	362	Fibrosis
2	9, F	Hepatic	0.057	12011-a	–	Present	281	Cirrhosis
3	9, M	Hepatic	0.05	385	–	Present	862	Fibrosis
4	9, M	Family history	0.32	767	–	Absent	131	CAH
5	11, M	Hepatic	Low1-153	380	–	Present	221	Cirrhosis
6	11, M	Hepatic	0.12	795	C/W WD	Present	5	Cirrhosis
7	12, M	Neurological	Low1-153	804	–	Present	4	Cirrhosis
8	13, M	Hepatic	0.176	546	–	Present	7	Cirrhosis
9	14, F	Hepatic	0.44	1787	–	Present	70001-e	Cirrhosis/CAH
10	16, M	Hepatic	0.35	7991-b	–	Present	48121-e	Cirrhosis
11	17, F	Neurological	Low1-153	680	–	Present	4	Cirrhosis
12	19, M	Neurological	Low1-153	782	–	Present	5	Cirrhosis
13	20, F	Hepatic	0.06	1700	–	Present	150	Fibrosis/CAH
14	22, M	Haemolysis	0.052	530	C/W WD	Present	1300	CAH
15	23, M	Hepatic	Low1-153	306	–	Present	56761-e	Fibrosis
16	27, M	Family history	Low1-153	914	–	Absent	55	Fibrosis
17	29, F	Family history	Low1-153	831	–	Absent	2	Fibrosis
18	30, F	Family history	Low1-153	478	–	Absent	474	Fibrosis
19	34, M	Hepatic	0.38	491-c	C/W WD	Absent	250	Cirrhosis
20	45, M	Hepatic	0.30	6341-d	–	Absent	2	Cirrhosis
21	47, M	Hepatic	0.15	516	–	Present	325	Fibrosis/CAH
22	58, F	Hepatic	0.37	1037	C/W WD	Absent	44	Fibrosis/CAH

↵1-150 Normal caeruloplasmin 0.20–0.45 g/l;
↵1-151 normal liver copper 15–60 μg/g dry weight liver;
↵1-152 normal urine copper less than 100 μg/24 h;
↵1-153 caeruloplasmin quantitated in optical density units.
↵1-a Liver copper quantitated at time of transplantation 12 months after presentation;
↵1-b liver copper quantitated at time of transplantation three years after presentation;
↵1-c liver copper quantitated at time of transplantation six years after presentation;
↵1-d liver copper quantitated at time of transplantation six months after presentation;
↵1-e urine copper quantitated while on penicillamine therapy.
C/W WD, radiocopper study consistent with the diagnosis of Wilson's disease; CAH, chronic active hepatitis; FHF, fulminant hepatic failure.