ESPN 53rd Annual Meeting

ESPN 2021


 
Liver and kidney transplantation in primary hyperoxaluria type 1
MARIA ARENA 2 RAFFAELLA LABBADIA 1 ISABELLA GUZZO 1 FRANCESCO EMMA 1 LUCA DELLO STROLOGO 1

1- Nephrology, dialysis and renal transplantation, Bambino Gesù children’s research hospital. IRCCS Rome, Italy
2- Università Cattolica del Sacro Cuore, Rome, Italy Nephrology, dialysis and renal transplantation, Bambino Gesù children’s research hospital. IRCCS Rome, Italy
 
Introduction:

Primary hyperoxaluria type 1 is a rare autosomal recessive disease caused by mutation of the AGTX gene responsible for derangement of hepatic AGT enzyme. It accounts for 1 to 2% of cases of pediatric end-stage kidney disease (ESKD).  

Aim of the study was to evaluate the outcomes of patients with primary hyperoxaluria who received combined or sequential liver-kidney transplantation.

Material and methods:

We retrospectively evaluated 10 patients with oxalosis who received a liver-kidney transplantation, with a median age of 5.6 years (range: 2.2-17.0) at kidney transplant. Five patients received a combined transplant. In five, the kidney transplant was postponed by a median time of 8 months (range 4-20) to achieve a lower oxalate burden for the graft. One of the patient died just after intervention and was excluded from further analysis. Median follow up was 3.2 years (range 1.6-11). All patients were on hemodialysis treatment at transplantation.

Results:

Median eGFR at 6 and at 12 month was 81.2 (range: 45.7–108.8) and 79.3 ml/min/1.73m2 (range 54.7 – 112.1) in patients who underwent combined transplant, and 45.7 (range 34.5–86.7) and 38.3 ml/min/1.73m2 (range: 29.9–77.5) in those with sequential transplant (p:NS).

In children with sequential transplant, serum oxalate before liver transplantation compared to pre-kidney transplant values showed a median drop of 51% (range 14-73%).

Sixth-month protocol biopsy showed the presence of oxalate crystals in all patients except one, demonstrating the recurrence of deposition in spite of the delay between liver and kidney transplant. 

Conclusions:

Liver and kidney transplant is currently the only possible option in patients with oxalosis. A delay between liver and kidney transplant does not appear to improve the final prognosis.