Table 1. Characteristics of Study Cohort of 219 subjects with BA.
| Characteristic | N (%) |
|---|---|
|
| |
| # patients | 219 |
|
| |
| Sex | |
| -male | 95 (43.4) |
| -female | 124 (56.6) |
|
| |
| Race | |
| -White | 139 (63.5) |
| -Black | 31 (14.1) |
| -Asian | 23 (10.5) |
| -Other | 26 (11.9) |
|
| |
| Age at Kasai (days) | |
| - N | 183 |
| - Mean ± SD | 56.4 ± 23.19 |
| - Median (Range) | 56 (7–125) |
|
| |
| Current age at evaluation (years) | |
| - N | 219 |
| - Mean ± SD | 10.5 ± 3.98 |
| - Median (Range) | 9.7 (5.1–17.9) |
|
| |
| Polysplenia syndrome | 5 (2.3) |
|
| |
| Other congenital malformations present | |
| -Cardiac | 23 (10.5%) |
| -Gastrointestinal | 25 (11.4%) |
|
| |
| Biochemical* characteristics at enrollment; median (IQR) | |
| -TB, mg/dL | 0.8 (0.4–1.2) |
| -AST, IU/L | 55 (38–99) |
| -ALT, IU/L | 54 (30–98) |
| -Alb, g/dL | 4.2 (3.8–4.5) |
| -INR | 1.1 (1.0–1.2) |
| -platelet count, ×109/L | 138 (85–209) |
| -GGT, IU/L | 67 (29–161) |
*
Normal liver laboratory values were defined as: serum total bilirubin (TB) ≤ 1.5 mg/dL, aspartate aminotransferase (AST) ≤45 IU/L, alanine aminotransferase (ALT) ≤ 40 IU/L, γ-glutamyltranspeptidase (GGT) ≤ 55 U/L, albumin ≥ 3.3 6g/dL, INR ≤ 1.3, and platelet count >150 × 109/L