ESPN 54th Annual Meeting

ESPN 2022


 
Isolated kidney transplantation under lumasiran therapy in primary hyperoxaluria type 1 (PH1): a report on 3 cases
ANNE-LAURE SELLIER-LECLERC 1 CHARLENE LEVI 1 CECILE ACQUAVIVA-BOURDAIN 1 STEPHANIE CLAVE 2 JUSTINE BACCHETTA 1

1- HOSPICES CIVILS DE LYON
2- ASSITANCE PUBLIQUE HOPITAUX DE MARSEILLE
 
Introduction:

 The RNA-interference therapy lumasiran demonstrated its efficacy to decrease urinary (UOx/creat) and circulating (POx) oxalate levels in PH1. Whether combined liver/kidney transplantation (CLKTx) can be replaced by isolated KTx and lumasiran remains debatable.

Material and methods:

 Three cases of genetically-confirmed PH1 patients receiving isolated KTx are described. They all received post-operatively “standard of care” (SOC), associating hyperhydration (3L/m²/day), potassium citrate (250mg/kg/day), pyridoxine and lumasiran.

Results:

 Patient 1: diagnosis 1.5 years, dialysis initiation 0.5 years, POx 110µmol/L (N<5) at the beginning of lumasiran at 2.5 years, KTx 13 months after lumasiran (POx 53µmol/L), deceased donor. Post-operative management: 3 early “prophylactic” hemodialysis sessions, then SOC. ARF on JJ obstruction at day 5, 15 hemodialysis sessions. At one month, renal function 125mL/min/1.73 m², POx 14µmol/L, UOx/creat 519µmol/mmol (<100). Follow-up 3 months, stable renal function, POx and UOx/creat.

Patient 2: diagnosis 17 years, dialysis initiation 23 years, POx 20µmol/L at the beginning of lumasiran at 26 years, KTx 10 months after lumasiran (POx 10µmol/L), living donor. No delayed graft function. Post-operative management: SOC. At one month, renal function 48mL/min/1.73 m², POx<5 µmol/L, UOx/creat 67µmol/mmol (<80). Follow-up 3 months, stable renal function and normal UOx/creat.

Patient 3: diagnosis 6 years, dialysis initiation 12 years, POx 128µmol/L at the beginning of lumasiran at 17 years, KTx 17 months after lumasiran (POx 23µmol/L), deceased donor. No delayed graft function. Post-operative management: SOC. At one month, renal function 50mL/min/1.73 m², POx 28µmol/L, UOx/creat 245µmol/mmol (<80). Arterial thrombosis post-lymphocele 41 days post KTx, requiring 13 daily hemodialysis sessions. Follow-up 3 months, renal function 50mL/min/1.73m² and stable UOx/creat.

Conclusions:

 We report the first successful isolated KTx in PH1 patients under lumasiran. Long-term data are obviously required. As described in CLKTx, post-operative hyperhydration and alkalinization is crucial, as long as urinary oxalate remains elevated from bone release.