| Title: | Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1 |
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| Authors: | ID Loos, Sebastian (Author)
ID Kemper, Markus J. (Author)
ID Schmaeschke, Kaja (Author)
ID Herden, Uta (Author)
ID Fischer, Lutz (Author)
ID Hoppe, Bernd (Author)
ID Kersnik-Levart, Tanja (Author)
ID Grabhorn, Enke (Author)
ID Schild, Raphael (Author)
ID Oh, Jun (Author), et al. |
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| Files: |  PDF - Presentation file, download (771,02 KB)
MD5: DE9F4180A62CE1CD34F9648D85D1C091
 URL - Source URL, visit https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2023.1157215/full
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| Language: | English |
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| Typology: | 1.01 - Original Scientific Article |
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| Organization: |  UKC LJ - Ljubljana University Medical Centre
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| Abstract: | 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. |
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| Keywords: | hyperoxaluria, infantile, juvenile, transplantation, outcome |
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| Publication status: | Published |
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| Publication version: | Version of Record |
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| Year of publishing: | 2023 |
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| Number of pages: | str. 1-8 |
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| Numbering: | Vol. 11, iss. [article no.] 1157215 |
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| PID: | 20.500.12556/DiRROS-28136  |
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| UDC: | 616-053.2 |
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| ISSN on article: | 2296-2360 |
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| DOI: | 10.3389/fped.2023.1157215 |
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| COBISS.SI-ID: | 243979267 |
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| Note: | Nasl. z nasl. zaslona;
Opis vira z dne 28. 7. 2025;
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| Publication date in DiRROS: | 10.03.2026 |
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| Views: | 26 |
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| Downloads: | 8 |
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