20.500.12556/DiRROS-28136 Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1 Introduction: Combined or sequential liver and kidney transplantation (CLKT/SLKT) restores kidney function and corrects the underlying metabolic defect in children with end-stage kidney disease in primary hyperoxaluria type 1 (PH1). However, data on long-term outcome, especially in children with infantile PH1, are rare. Methods: All pediatric PH1-patients who underwent CLKT/SLKT at our center were analyzed retrospectively. Results: Eighteen patients (infantile PH1 n=10, juvenile PH1 n = 8) underwent transplantation (CLKT n=17, SLKT n = 1) at a median age of 5.4 years (1.5–11.8). Patient survival was 94% after a median follow-up of 9.2 years (6.4–11.0). Liver and kidney survival-rates after 1, 10, and 15 years were 90%, 85%, 85%, and 90%, 75%, 75%, respectively. Age at transplantation was significantly lower in infantile than juvenile PH1 (1.6 years (1.4–2.4) vs. 12.8 years (8.4–14.1), P = 0.003). Median follow-up was 11.0 years (6.8–11.6) in patients with infantile PH1 vs. 6.9 years (5.7–9.9) in juvenile PH1 (P = 0.15). At latest follow-up kidney and/or liver graft loss and/or death showed a tendency to a higher rate in patientswith infantile vs. juvenile PH1 (3/10 vs. 1/8, P=0.59). Discussion: In conclusion, the overall patient survival and long-term transplant outcome of patients after CLKT/SLKT for PH1 is encouraging. However, results in infantile PH1 tended to be less optimal than in patients with juvenile PH1. hyperoxaluria infantile juvenile transplantation outcome true false true Angleški jezik Ni določen Neznano 2026-03-10 15:02:34 2026-03-10 15:02:34 2026-03-11 03:59:37 0000-00-00 00:00:00 2023 0 0 Nasl. z nasl. zaslona; Opis vira z dne 28. 7. 2025; str. 1-8 iss. [article no.] 1157215 Vol. 11 2023 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 616-053.2 2296-2360 10.3389/fped.2023.1157215 243979267 RAZ_Loos_Sebastian_2023.pdf RAZ_Loos_Sebastian_2023.pdf 1 DE9F4180A62CE1CD34F9648D85D1C091 dd0eaa5bc981eedd228f10d8756da74d0c03e49c4678f3042e554c7cf234b213 f1cd8b08-1c89-11f1-acf8-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=41594 https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2023.1157215/full 0 fbff8ce1-1c89-11f1-acf8-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=41595 Univerzitetni klinični center Ljubljana 0 0 0