Table 1.
Clinical characteristics of domino liver donors and recipients
| DLT recipient | Genetic mutation | Post DLT Follow up time (months) | Age (year) and weight (kg) at time of DLT | Post‐op complications | Mean posttransplant BCAA levels (μmol/L; mean ± sd) | Mean posttransplant day for BCAA assessment (range) | Current nutritional status | Current status, ALT/ GGT (IU/L) | |||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Non‐MSUD recipient | MSUD donor | Isoleucine | Leucine | Valine | |||||||
| PA | PCCA p. G117D homozygous | 36 | 11.7/39 | 5.1/21 | Hepatic artery thrombosis, TCMR, EBV hepatitis | 68 ± 40 | 101 ± 56 | 155 ± 73 | 94 (1‐272) | Regular diet with Propimex‐2 | Alive, 42/34 |
| CN‐1 | UGT1A1 c.222c>a (p. y74*) | 34 | 13.8/64 | 29.1/68 | TCMR | 85 ± 25 | 126 ± 33 | 217 ± 71 | 175 (7‐342) | Regular diet | Alive, 25/31 |
| CN‐1 | NA | 27 | 20.7/57 | 18/69 | TCMR | 64 | 110 | 198 | 450 | Regular diet | Alive, 12/7 |
| CPSD‐1 | CPS1 c.306_311 dup and GAA TGG and c.4022 del G | 23 | 1.4/11, received left lateral segment graft | 27.1/80 | TCMR Steatosis, CMV hepatitis |
34 ± 15 | 50 ± 14 | 89.5 ± 21 | 7 (1‐16) | Regular diet | Alive, 55/15 |