Table 2.
Metabolic parameters for OTC deficiency patients before and after liver transplantation.
| Patient No | Ammonia at diagnosis (μmol/L) |
Number of hospital admissions from diagnosis to LT | Number of hospital admissions with hyperammonemia (≥100uM) from diagnosis to LT | Peak ammonia levels during the follow up (μmol/L) |
Pre-LT natural/total protein intake (g/kg/day) |
Age of LT (m) |
LT Centre | Procedure | Follow up period | Post-LT protein intake |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 176 | Multiple | Multiple | 150 | 0.85/1 | 156 | LTH | LDLT | 11y | NR |
| 2 | N/A | 10 | 3 | 165 | 0.6/0.6 | 152 | KCLH | DDLT | 7y | NR |
| 3 | 264 | 3 | 1 | 105 | 1/1 | 35 | KCLH | DDLT | 5y, re-transplanted at 19d | NR |
| 4 | 249 | Multiple | 10 | 230 | 1/1 | 77 | BCH | DDLT | 12y, re-transplanted at 79 m | NR |
| 5 | 339 | Multiple | 3 | 212 | 1.5/1.5 | 56 | BCH | DDLT | 9y | NR |
| 6 | 84 | Multiple | 0 | 72 | 1.5/1.5 | 41 | BCH | DDLT | 9y | NR |
| 7 | 81 | 10 | 7 | 289 | 1/1 | 71 | BCH | DDLT | 6 h, deceased | NR |
| 8 | 207 | 6 | 5 | 168 | 1.5/1.5 | 70 | BCH | DDLT | 1.5y | NR |
| 9 | 249 | Multiple | Multiple | 200 | 1.5/1.5 | 79 | BCH | DDLT | 12y | NR |
| 10 | N/A | 3 | 0 | 61 | 1.2/1.2 | 35 | KCLH | DDLT | 9y | NR |
| 11 | N/A | 3 | 2 | 289 | 1.5/1.5 | 62 | BCH | DDLT | 13y | NR |
| 12 | 47 | 1 | 0 | <100 | 1.5/1.5 | 39 | BCH | LDLT | 3y | NR |
| 13 | 300 | Multiple | 0 | 80–90 | 1.1/1.5 | 119 | KCLH | DDLT | 12y | NR |
| 14 | 220 | 22 | 15 | 312 | 0.6/1 | 51 | KCLH | DDLT | 4y | NR |
| 15 | 1354 | 10 | 3 | 140 | 0.8/1.1 | 10 | KCLH | LDLT | 2.5y | NR |
| 16 | 1069 | Multiple (Received 5 hepatocyte infusions before LT) |
2 | 116 | 1.2/1.8 | 6 | KCLH | DDLT | 11y | NR |
| 17 | 1800 | 4 | 0 | 68 | 1.45/1.58 | 7 | KCLH | DDLT | 4y | NR |
| 18 | 1800 | 13 | 6 | 2800 | 0.5/0.9 | 11 | KCLH | LDLT | 3y | NR |
| 19 | 1000 | 4 | 2 | 294 | 1/1 | 24 | KCLH | DDLT | 1y | NR |
| 20 | 2808 | 2 | 0 | 92 | 1.14/1.14 | 14 | KCLH | LDLT | 1y | NR |
LT: Liver transplantation; Multiple: ≥5; LTH: Leeds Teaching Hospitals; KCLH: Kings College Hospital; BCH: Birmingham Children's Hospital; LDLT: Living related donor liver transplantation; DDLT: Deceased donor liver transplantation; h: hours; d: days; m: Months; y: Years; NR: Not restricted.