Table 2.
Suggestions for organ transplantation strategies in PH1 patients according to residual GFR (ml/min per 1.73 m²), systemic involvement, and local facilities (from [68]).
| Tx strategy | Combined simultaneous liver + kidney | Liver first Kidney as a second step | Isolated kidney | Isolated liver |
|---|---|---|---|---|
| HD strategy | Peroperative ± postoperative according to Pox and GFR | Standard HD following liver Tx aiming at Pox <20 μmol/L | Pre- and peroperative | Sometimes peroperative |
| 40 < GFR < 60 | No | No | No | Optional |
| 20 < GFR < 40 | Yes | No | (1) In developing countries (2) In very selected patients |
No |
| GFR < 20 | Yes | Yes | No | No |
| Infantile form (ESRD <2 years) |
Yes | Yes (emergency) | No | No |
Abbreviations: ESRD end-stage renal disease; GFR glomerular filtration rate; HD hemodialysis; Pox plasma oxalate; Tx transplantation.