Table 1

Clinical Data OF Patients with AGS, FIC1 and BSEP Disease



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

T Bili = Total Bilirubin. Non-responders*.

Emerick et al. BMC Gastroenterology 2008 8:47   doi:10.1186/1471-230X-8-47