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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*.