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
No
No
No
Optional
Yes
No
(1) In developing countries (2) In very selected patients
No
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.
