Pediatric Wilson disease presenting as acute liver failure: Prognostic indices

Table 2 Demographics and baseline clinical characteristics in patients with Wilson disease with acute liver failure with non-transplanted survival (n = 35), poor outcome (n = 6; 3 dying, 3 surviving after liver transplantation), and dying (n = 3)

	Poor outcome group	Survival group	P value	Dying group	Survival group	P value
	n = 6 (14.63%)	n = 35 (85.37%)		n = 3 (7.89%)	n = 35 (92.10%)
Males (%)	2 (33.33%)	20 (57.14%)	0.390	1 (33.33%)	20 (57.14%)	0.577
Age (yr)	9.82 ± 2.16	10.54 ± 2.60	0.685	11.28 ± 2.17	10.54 ± 2.64	0.465
Jaundice	6 (100%)	26 (74.29%)	0.309	3 (100%)	26 (74.29%)	1.000
Hepatomegaly	2 (33.33%)	8 (22.86%)	0.622	1 (33.33%)	8 (22.86%)	1.000
Splenomegaly	2 (33.33%)	13 (37.14%)	1.000	1 (33.33%)	13 (37.14%)	1.000
Encephalopathy	4 (66.67%)	3 (8.57%)	0.005a	2 (66.67%)	3 (8.57%)	0.040a
Total bilirubin (μmol/L)	452.22 ± 294.19	129.20 ± 175.64	0.007a	395.03 ± 187.16	129.20 ± 175.64	0.021a
Direct bilirubin (μmol/L)	251.27 ± 157.76	75.79 ± 108.91	0.008a	229.77 ± 115.63	75.79 ± 108.91	0.025a
Bile acid (μmol/L)	103.80 ± 96.52	88.61 ± 46.64	0.650	128.87 ± 129.41	88.61 ± 46.64	0.976
ALT (IU/L)	48.67 ± 43.32	61.68 ± 49.13	0.519	74.67 ± 47.44	61.68 ± 49.13	0.432
AST (IU/L)	89.00 ± 26.01	131.24 ± 105.11	0.555	111.67 ± 12.22	131.24 ± 105.11	0.607
GGT (IU/L)	89.67 ± 56.39	159.63 ± 76.06	0.035a	51.00 ± 37.27	159.63 ± 76.06	0.019a
ALP (IU/L)	155.33 ± 147.72	224.19 ± 150.24	0.285	220.67 ± 188.32	224.19 ± 150.24	0.935
Albumin (g/L)	26.93 ± 6.67	25.74 ± 4.23	0.658	28.80 ± 9.20	25.74 ± 4.23	0.343
Total protein (g/L)	56.10 ± 7.80	60.81 ± 7.93	0.249	52.50 ± 5.75	60.81 ± 7.93	0.095
Ammonia (μmol/L)	96.33 ± 26.96	62.65 ± 27.94	0.011a	114.33 ± 25.58	62.65 ± 27.94	0.015a
Serum creatinine (μmol/L)	55.00 ± 47.44	42.24 ± 13.45	0.791	26.33 ± 1.53	42.24 ± 13.45	0.030a
Serum urea nitrogen (mmol/L)	7.75 ± 5.96	3.94 ± 1.20	0.076	4.43 ± 0.95	3.94 ± 1.20	0.386
PT (s)	39.00 ± 15.36	27.04 ± 7.06	0.011a	48.4 ± 17.84	27.04 ± 7.06	0.009a
INR	4.12 ± 2.20	2.53 ± 0.89	0.012a	5.46 ± 2.57	2.53 ± 0.89	0.009a
APTT (s)	74.17 ± 27.70	55.85 ± 12.80	0.042a	90.00 ± 34.01	55.85 ± 12.80	0.028a
Fibrinogen (g/L)	1.03 ± 0.40	1.40 ± 0.41	0.080	0.733 ± 0.23	1.40 ± 0.41	0.014a
WCC × 109/L	11.40 ± 6.54	8.15 ± 4.67	0.203	6.40 ± 2.36	8.15 ± 4.67	0.626
Hemoglobin (g/L)	85.47 ± 19.42	106.41 ± 22.89	0.027a	94.73 ± 19.40	106.41 ± 22.89	0.316
Reticulocyte %	7.78 ± 3.81	4.38 ± 2.72	0.080	7.05 ± 4.10	4.38 ± 2.72	0.136
Platelet count × 109/L	100.33 ± 41.32	109.66 ± 48.01	0.796	69.33 ± 11.59	109.66 ± 48.01	0.129
Serum sodium (mmol/L)	133.83 ± 2.48	136.28 ± 3.28	0.073	132.00 ± 2.00	136.28 ± 3.28	0.029a
Ceruloplasmin (g/L)	0.080 ± 0.002	0.068 ± 0.029	0.082	0.079 ± 0.00	0.068 ± 0.029	0.281
24 h urine copper (μg/24 h)	2563 ± 1915	2083 ± 1964	NA	582.00 ± NA	2083 ± 1964	NA
KCHC	12.00 ± 1.67	8.43 ± 3.42	0.020a	11.33 ± 0.58	8.43 ± 3.42	0.157
MELD/PELD score	31.67 ± 9.87	19.00 ± 7.65	0.007a	38.00 ± 7.00	19.00 ± 7.65	0.006a
LIU-PT score	223.33 ± 63.49	115.77 ± 54.29	0.002a	221.67 ± 45.18	115.77 ± 54.29	0.016a
LIU-INR score	390.67 ± 89.19	217.49 ± 95.36	0.002a	424.33 ± 110.21	217.49 ± 95.36	0.019a
aLIU-PT score	338.50 ± 114.04	160.31 ± 79.75	0.003a	353.00 ± 35.38	160.31 ± 79.75	0.010a
aLIU-INR score	428.17 ± 147.81	189.74 ± 101.95	0.002a	467.00 ± 61.25	189.74 ± 101.95	0.009a
Devarbhavi model score	32.13 ± 18.64	8.59 ± 11.62	0.007a	30.47 ± 10.00	8.59 ± 11.62	0.014a

^aP value < 0.05. aLIU: Admission to Liver Injury Unit; ALP: Alkaline phosphatase; ALT: Alanine aminotransferase; APTT: Active partial thromboplastin times; AST: Aspartate aminotransferase; GGT: Gamma-glutamyl transpeptidase; INR: International normalized ratio; KCHC: King’s College Hospital Criteria; LIU: Liver Injury Unit; MELD/PELD: Model for end-stage liver disease/pediatric end-stage liver disease; NA: Not available; PT: Prothrombin time; WCC: White cell count.