Sequential Versus Combined Liver-Kidney Transplantation in Pediatric Primary Hyperoxaluria Type 1: A Multi-Center...

Combined liver-kidney transplantation is the standard treatment for pediatric primary hyperoxaluria Type 1 with advanced kidney disease, but sequential liver-kidney transplantation is increasingly used where living-donor transplantation predominates. Direct comparisons between these strategies remain limited.

We retrospectively analyzed 19 pediatric patients with chronic kidney disease Stage 4 or higher who completed combined (n = 10) or sequential (n = 9) liver-kidney transplantation for primary hyperoxaluria Type 1 across four countries. The comparative analysis was restricted to patients who received both organs; patients who underwent liver transplantation without subsequent kidney transplantation were excluded.

Patients who completed sequential transplantation had earlier onset of primary hyperoxaluria Type 1-related symptoms (3.0 vs. 12.0 months, p = 0.029) and lower estimated glomerular filtration rate at liver transplantation (16.5 vs. 36.5 mL/min/1.73 m², p = 0.043) than those who completed combined transplantation. Among recipients who completed both procedures, major perioperative complications and patient survival did not differ significantly; no perioperative deaths occurred after the liver transplantation stage among patients who subsequently completed sequential transplantation. Death-censored kidney graft survival was higher in the completed-sequential group (log-rank p = 0.045). Among patients who completed sequential transplantation, an interval exceeding 6 months between liver and kidney transplantation showed a descriptive trend toward better preserved kidney function.

Among pediatric patients with primary hyperoxaluria Type 1 who completed both liver and kidney transplantation, sequential transplantation was associated with favorable observed kidney graft outcomes. These findings describe outcomes among recipients who completed both procedures and should not be interpreted as outcomes of an initially intended sequential strategy.