Chronic liver disease is universal in children with biliary atresia living with native liver

Table 1 Clinical and laboratory characteristics of the 52 patients with biliary atresia surviving with native livers after Kasai surgery

Characteristic		n (%)
Sex	Male	20 (38.4)
	Female	32 (61.6)
Ethnicity	Chinese	32 (61.5)
	Malays	15 (28.8)
	Indians	5 (9.6)
Age at Kasai surgery in d	mean ± SD	65.5 ± 26.3
	Median	60
	Range	30-148
Kasai surgery ≤ 60 d	Yes	30 (57.7)
	No	22 (42.3)
Age at latest follow-up in yr	mean ± SD	8.3 ± 6.1
	Median	7.4
Medical conditions present	No	46 (88.5)
	Yes	6 (11.5)
	Congenital anomalies	3 (5.7)
Presence of failure to thrive
All, n = 52	Yes	14 (26.9)
	No	38 (73.1)
< 5 yr old, n = 17	Yes	3 (17.6)
	No	14 (82.4)
≥ 5 yr old, n = 35	Yes	11 (31.4)
	No	24 (68.6)
Short stature
All, n = 51	Yes	10 (19.6)
	No	41 (80.4)
< 5 yr old, n = 16	Yes	1 (5.9)
	No	16 (94.1)
≥ 5 yr old, n = 32	Yes	9 (26.5)
	No	25 (73.5)
Laboratory indices at review	White cell count, × 109/L	6.2 (4.8)
Median (IQR)	Platelet count, × 109/L	131 (169)
	Total bilirubin, μmol/L	18 (39)
	Serum albumin, g/L	41 (8)
	International normalised ratio	1.1 (0.1)
	Alanine transferase, IU/L	54 (64)
	Aspartate transferase, IU/L	70 (80)
	Gamma glutamyl-transpeptidase, IU/L	109 (174)

Congenital anomalies present: One each for aortic anomalies, atrial septal defect and craniosynostosis. IQR: Interquartile range.

Table 2 Medical status of the 52 patients with biliary atresia surviving with native livers after Kasai surgery n (%)

Medical variable	Data available, n	All, n = 52	< 5 yr old, n = 17	≥ 5 yr old, n = 35	P value,
					< 5 yr old vs ≥ 5 yr old
Failure to thrive, weight-for-age z-score < 2 SD	52	14 (26.9)	3 (17.6)	11 (31.4)	0.29
Short stature, height-for-age z-score < 2 SD	51	10 (19.6)	1 (5.9)	9 (26.5)	0.089
Medical complications
Portal hypertension	52	21 (40.4)	2 (11.8)	19 (54.3)	0.034
Variceal bleeding	52	13 (25.0)	1 (5.8)	12 (34.3)	0.026
Ascites	52	3 (5.8)	2 (11.8)	1 (2.9)	0.20
Cholangitis	52	19 (36.3)	8 (47.1)	11 (31.4)	0.27
Portopulmonary hypertension	52	2 (3.8)	0 (0)	2 (5.7)	0.31
Hepatopulmonary syndrome	52	0 (0)	0 (0)	0 (0)	-
Bone fracture	52	0 (0)	0 (0)	0 (0)	-
Laboratory indices
White cell count, < 4 × 109/L	49A	13 (25.0)	1 (5.9)	13 (40.6)	0.017
Platelet count, < 150 × 109/L	50A	28 (53.8)	8 (47.1)	22 (66.7)	0.28
Total bilirubin, ≥ 17 μmol/L	52	26 (50.0)	6 (35.3)	21 (60.0)	0.09
Albumin, < 35 g/L	52	6 (11.5)	1 (5.8)	5 (14.3)	0.37
International normalized ratio, ≥ 1.3	52	12 (23.1)	5 (29.4)	7 (20.0)	0.70
Alanine transferase, ≥ 40 IU/L	52	35 (67.3)	13 (76.5)	24 (68.6)	0.56
Aspartate transferase, ≥ 40 IU/L	52	38 (73.1)	15 (88.2)	25 (71.4)	0.18
Gamma glutamyl-transpeptidase, ≥ 55 IU/L	52	36 (69.2)	11 (64.7)	27 (77.1)	0.34
Absence of any medical complications	52	15 (28.8)	6 (35.3)	9 (25.7)	0.98
Absence of abnormal laboratory indices	49	3 (6.1)	1 (5.9)	2 (6.3)	0.47
Ideal medical outcome	49	1 (2.0)	0 (0)	1 (2.9)

A: Data for white cell count for 3 patients and platelet count for 2 patients were not available for review. Ideal medical outcome was defined as an absence of any medical complications and presence of normal laboratory indices.