ESPN 50th Annual Meeting

ESPN 2017


 
Combined liver-kidney transplantation in children with autosomal recessive polycystic kidney disease (ARPKD) – national center experience
GRENDA RYSZARD 1 JARMUZEK WIOLETTA 1 JANKOWSKA IRENA 1 RUBIK JACEK 1 KALICINSKI PIOTR 1

1- THE CHILDREN’S MEMORIAL HEALTH INSTITUTE; WARSAW, POLAND
 
Introduction:

Autosomal recessive polycystic kidney disease (ARPKD), depending on gene expression, may be presented as the multiorgan disease, with liver involvement. In particular cases combined liver-kidney transplantation (CLKTx) is necessary. Purpose of this presentation was the summary of single center experience with 16 CLKTxs performed in 2000-2015.

Material and methods:

Patients and methods: overall 16 patients (8 boys and 8 girls; at the mean age of 13 years) received liver and kidney transplant due to ARPKD. In 3 cases the renal transplantation ( combined with liver) was the second transplantation after loss of the previous (isolated) renal graft. Overall 14 (of 16) patients received monoclonal blocking induction (anti-IL 2Rα moab; daclizumab n=13; basiliximab n=1) and maintenance immunosupression incuded: - Pred+TAC (n= 9); - MMF+TAC (n= 3) and Pred+MMF+TAC (n=2) . Patients with no induction (n=2) were on triple therapy: Pred+MMF+TAC.

Results:

Results: There was no episode of acute rejection in renal and a single episode in liver transplants within 5-year follow-up. Renal function was good at two-years post-Tx (Schwartz mean eGFR 72,16 mL/min/1.73m2); in 3 cases eGFR deteriorated at 3-years post-Tx (mean eGFR 55 mL/min/1.73m2 ) and in 5 after 5 years (mean eGFR 53.6 mL/min/1.73m2); as a result of chronic allograft nephropathy with no signs of antibody-mediated rejection. Overall 1 and – 5 years patient survival was 94% and kidney-liver graft survival was 100%.

Conclusions:

Conclusion: - long-term outcome of combined liver-kidney transplantation in children was very satisfactory, with no acute rejection, however chronic calcineurine- inhibitor related nephropathy developed with time in a half of these patients.