1. Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1Sebastian Loos, Markus J. Kemper, Kaja Schmaeschke, Uta Herden, Lutz Fischer, Bernd Hoppe, Tanja Kersnik-Levart, Enke Grabhorn, Raphael Schild, Jun Oh, 2023, izvirni znanstveni članek Povzetek: 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.
Ključne besede: hyperoxaluria, infantile, juvenile, transplantation, outcome
Objavljeno v DiRROS: 10.03.2026; Ogledov: 130; Prenosov: 105
 Celotno besedilo (771,02 KB)
Gradivo ima več datotek! Več... |
