In:
Pediatric Nephrology, Springer Science and Business Media LLC, Vol. 36, No. 8 ( 2021-08), p. 2217-2226
Abstract:
Primary hyperoxaluria type 1 (PH1) is characterized by hepatic overproduction of oxalate and often results in kidney failure. Liver-kidney transplantation is recommended, either combined (CLKT) or sequentially performed (SLKT). The merits of SLKT and the place of an isolated kidney transplant (KT) in selected patients are unsettled. We systematically reviewed the literature focusing on patient and graft survival rates in relation to the chosen transplant strategy. Methods We searched MEDLINE and Embase using a broad search string, consisting of the terms ‘transplantation’ and ‘hyperoxaluria’. Studies reporting on at least four transplanted patients were selected for quality assessment and data extraction. Results We found 51 observational studies from 1975 to 2020, covering 756 CLKT, 405 KT and 89 SLKT, and 51 pre-emptive liver transplantations (PLT). Meta-analysis was impossible due to reported survival probabilities with varying follow-up. Two individual high-quality studies showed an evident kidney graft survival advantage for CLKT versus KT (87% vs. 14% at 15 years, p 〈 0.05) with adjusted HR for graft failure of 0.14 (95% confidence interval: 0.05–0.41), while patient survival was similar. Three other high-quality studies reported 5-year kidney graft survival rates of 48–89% for CLKT and 14–45% for KT. PLT and SLKT yielded 1-year patient and graft survival rates up to 100% in small cohorts. Conclusions Our study suggests that CLKT leads to superior kidney graft survival compared to KT. However, evidence for merits of SLKT or for KT in pyridoxine-responsive patients was scarce, which warrants further studies, ideally using data from a large international registry.
Type of Medium:
Online Resource
ISSN:
0931-041X
,
1432-198X
DOI:
10.1007/s00467-021-05043-6
Language:
English
Publisher:
Springer Science and Business Media LLC
Publication Date:
2021
detail.hit.zdb_id:
1463004-7
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