Diagnostic criteria for acute liver failure due to Wilson disease

Table 1 Baseline characteristics at initial presentation

	Sex	Age(yr)	ALT(U/L)	AP(U/L)	Bilirubin(mg/dL)	INR	Creatinine(mg/dL)	U-Copper(μmol/24 h)	Grade ofHE	Bx-Copper(μg/g)	KFR	Mutation	NS	Outcome
FWD	F	46	19	118	8.5	2.7	2.20	411.00	2	ND	Yes	ND	4	OLT, alive
	F	16	45	122	10.6	2.0	0.60	104.00	1	1276	No	ND	5	Alive
	F	18	31	56	48.0	2.4	4.30	38.00	2	1500	ND	H1069Q/H1069Q	8	OLT, alive
	F	17	143	n.d.	15.0	1.6	1.00	60.00	2	ND	No	H1069Q/IVS4-1:g > a	7	Alive
	F	16	44	262	11.0	1.7	1.70	3.89	1	400	Yes	H1069Q/2299Ins	4	Alive
	F	18	64	193	52.0	2.6	0.77	25.00	1	1581	ND	T977M/T977M	7	OLT, alive
	F	10	22	19	13.0	1.9	0.60	11.93	1	ND	Yes	ND	5	Alive
										ALF etiology
ALF	F	52	3188	181	15.5	1.5	0.95	3.12	2	Fulminant hepatitis B				Alive
	F	85	386	99	3.8	2.6	1.18	5.56	3	Toxic (tuberculostatics)				Alive
	F	35	1442	111	23.2	1.5	0.50	2.23	2	Toxic (venlafaxin)				Alive
	F	34	1782	266	1.5	1.7	0.55	1.72	1	Unknown				Alive
	F	59	2301	59	4.0	1.8	1.25	0.80	4	Toxic (acetaminophen)				Death
	F	43	1285	142	9.4	1.8	0.58	4.22	2	Toxic (opipramol)				Alive
	M	18	2953	179	24.5	2.4	0.64	4.39	2	Fulminant hepatitis B				Alive
	M	32	2522	79	38.0	3.2	0.89	5.80	1	Unknown				OLT, alive

FWD: acute liver failure secondary to WD; ALF: acute liver failure; F: female; M: male; ALT: alanine aminotransferase, normal range below 34 and 50 for female and male, respectively; AP: alkaline phosphatase, normal range below 126 and 130 for female and male, respectively; HE: hepatic encephalopathy; Bx-Copper: hepatic copper content; KFR: Kayser-Fleischer ring; NS: Nazer-Score; OLT: orthotopic liver transplantation; ND: Not determined.

Table 2 Comparison of clinical findings between the groups

	FWD	ALF	P
Age (yr)	20.1 ± 11.7	44.8 ± 20.6	0.016
MELD score	27.6 ± 9.5	20.3 ± 7.0	0.108
HE (grade 1-4)	1.4 ± 0.5	2.13 ± 1.0	0.122
Serum ALT (U/L)	53 ± 43	1982 ± 938	< 0.0001
Serum AST (U/L)	87 ± 44	2756 ± 2941	0.037
AST-ALT ratio	2.3 ± 1.5	1.5 ± 1.2	0.266
Serum AP (U/L)	128 ± 89	142 ± 66	0.749
Serum LDH (U/L)	535 ± 638	1567 ± 2707	0.382
Serum bilirubin (mg/dL)	23 ± 19	15 ± 13	0.372
AP-bilirubin ratio	9.3 ± 8.9	32.5 ± 59.0	0.363
INR	2.1 ± 0.4	2.1 ± 0.6	0.816
Albumin (g/L)	29.8 ± 4.7	32.0 ± 3.8	0.379
CHE (kU/L)	1.8 ± 1.2	4.3 ± 1.2	0.005
Hemoglobin (g/L)	7.0 ± 2.2	12.6 ± 1.8	< 0.0001
Serum copper (μmol/L)	28.1 ± 29.4	17.8 ± 2.5	0.376
Serum ceruloplasmin (g/L)	0.12 ± 0.08	0.19 ± 0.03	0.07
Urinary copper (μmol/24 h)	93.4 ± 144.0	3.5 ± 1.8	0.001

FWD: acute liver failure secondary to WD; ALF: acute liver failure; HE: hepatic encephalopathy; ALT: alanine aminotransferase; AP: alkaline phosphatase; INR: international normalized ratio; CHE: choline esterase.