Successful treatment of acute liver failure due to Wilson’s disease: Serendipity or fortuity?

Table 1 Prognostic scores for acute liver failure due to Wilson’s disease applied to Case 1

Parameter	Value	New Wilson Index	Nazer score
Total bilirubin in mg/dL	14.7	3	3
INR	4.48	4	4
AST in IU/L	139	1	1
WBC as × 109/L	14.9	3	NA
Alb in g/dL	2.77	2	NA
Total score	NA	13	8

Alb: Albumin; AST: Aspartate aminotransferase; INR: International normalized ratio; NA: Not applicable; WBC: White blood cell.

Table 2 Laboratory characteristics over time for a boy with acute liver failure due to Wilson’s disease (Case 1)

Parameter	At onset	4 months	5 months	9 months	1 year	2 years	3 years	4 years	5 years
AST in IU/L	264	69	60	43	30	42	23	20	23
ALT in IU/L	300	102	59	30	21	29	19	19	25
Total bilirubin in mg/dL	14.5	1.21	0.59	0.99	1.37	0.82	0.46	0.90	1.10
Direct bilirubin in mg/dL	4.5	ND	0.22	0.38	0.49	0.31	0.22	0.37	0.55
GGT in IU/L	ND	38	31	29	16	16	17	19	16
ALP in IU/L	ND	344	717	750	413	413	429	430	190
CHE in IU/L	ND	3424	3955	5362	7890	7890	8357	7912	6054
Gamma globulin, %	34	17.9	13.2	14	12.6	15.5	14.9	12	13.2
INR	4.48	1.26	1.29	1.3	1.22	1.21	1.18	1.24	1.17
Platelets as × 103/μL	37	88	118	154	151	186	270	266	273
Ceruloplasmin in mg/dL	11	11	ND	ND	ND	9	5	6	0
24-hour UCE in µg	200	60	69	61	40.4	40.4	38.7	23.9	62.4

AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; GGT: Gamma-glutamyl transferase; ALP: Alkaline phosphatase; 24 hour-UCE: 24-hour urinary copper excretion; CHE: Cholinesterase; INR: International normalized ratio; ND: Not done.

Table 3 Prognostic scores for acute liver failure due to Wilson’s disease applied to Case 2

Parameter	Value	New Wilson Index	Nazer score
Total bilirubin in mg/dL	14.5	3	3
INR	3.8	4	4
AST in UI/L	50	0	0
WBC as × 109/L	14.7	3	NA
Alb in g/dL	2.4	3	NA
Total score	NA	13	7

Alb: Albumin; AST: Aspartate aminotransferase; INR: International normalized ratio; NA: Not applicable; WBC: White blood cell.