Table 1 Patient characteristics.

From: Upregulated beta-defensin-1 in murine and human biliary atresia associates with human native liver survival

Variable

Normal controls

TNC patients

BA patients

FCD patients

Overall p value

eBA

lBA

eFCD

lFCD

Number of samples

15

12

83

24

–

39

44

18

6

–

Male, n of total

11/15

9/12

45/83

14/24

–

21/39

24/44

12/18

2/6

–

Non-syndromic BA

–

–

34/39

41/44

–

–

–

Syndromic BA

–

–

5/39

3/44

–

–

–

ALGS

–

–

–

–

3/18

2/6

–

PFIC

–

–

–

–

15/18

4/6

–

Age at KPE or sample, d

560 (330–883)

48 (35–66)

51 (36–84)

213 (160–835)

804 (164–1817)

1404 (334–3533)

< 0.0001

Total bilirubin at biopsy, mg/dl

–*

8.0 (5.8–8.9)

9.9 (6.7–12.3)

6.9 (1.6–20.9)

5.8 (0.8–11.7)

17.3 (1.7–23.6)

< 0.0001

ALT at biopsy, U/l

–*

146 (83–225)

210 (154–254)

181 (89–466)

86 (60–499)

211 (75–229)

< 0.0001

AST at biopsy, U/l

–*

85 (48–128)

153 (101–249)

113 (51–257)

75 (30–236)

81 (58–101)

< 0.0001

GGT at biopsy, U/l

–*

176 (81–642)

439 (238–627)

152 (71–346)

43 (20–138)

197 (48–256)

< 0.0001

  1. Data are shown as frequencies or as medians with interquartile range. Overall p values were calculated using Kruskal-Wallis tests and are shown in the rightmost column; Dunn’s post-hoc comparisons are provided in Table S1. Abbreviations: ALGS, Alagille syndrome; ALT, alanine transaminase; AST, aspartate transaminase; BA, biliary atresia; eBA, early biliary atresia; eFCD, early familial cholestasis disease (PFIC or ALGS); FCD, familial cholestasis disease (PFIC or ALGS); GGT, gamma-glutamyl transferase; KPE, Kasai portoenterostomy; lBA, late biliary atresia; lFCD, late familial cholestasis disease (PFIC or ALGS); TNC, transient neonatal cholestasis.
  2. *An increase in bilirubin or liver enzymes was an exclusion criterion for normal controls.
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