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Table 1 Clinical Data OF Patients with AGS, FIC1 and BSEP Disease

From: Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion

   

Pre-PEBD Baseline

Post-PEBD

Pt

Disease

Age at PEBD

(yrs)

Itch

T Bili

(mg/dL)

GGT

(IU/L)

ALT

(IU/L)

Bile salts

(μM/L)

Age at F/U

(yrs)

Itch

T Bili

(mg/dL)

ALT

(IU)

Bile salts

(μM/L)

1

AGS

11.6

4

1.2

170

82

81

14

1

2.1

198

24

2

AGS

18

4

4.4

370

265

58

18.3

1

2.8

155

24

3

AGS

16

4

2.0

329

121

97

16.8

2

2.9

214

21

4

FIC1

0.5

3

8.9

32

97

97

1.5

1

0.6

270

6

5

FIC1

4.4

4

5.4

33

53

255

6.1

1

0.3

74

6

6

FIC1

4.8

4

10.8

23

40

340

5.8

1

6.9

68

120

7

FIC1 *

7.3

4

6.7

28

52

485

9

4

4.5

28

63

8

BSEP*

0.4

3

3.1

45

112

 

2.9

2

2

83

488

9

BSEP*

2.7

3

4.7

29

190

184

4.3

4

4.5

171

257

10

BSEP

4.5

2

4.1

79

173

225

5

1

0.8

85

32

  1. T Bili = Total Bilirubin. Non-responders*.