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 | Â | 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 |