Universität zu Köln

Metry, Elisabeth L., Garrelfs, Sander F., Peters-Sengers, Hessel ORCID: 0000-0003-3145-864X, Hulton, Sally-Anne, Acquaviva, Cecile, Bacchetta, Justine, Beck, Bodo B., Collard, Laure, Deschenes, Georges, Franssen, Casper ORCID: 0000-0003-1004-9994, Kemper, Markus J., Lipkin, Graham W., Mandrile, Giorgia, Mohebbi, Nilufar, Moochhala, Shabbir H., Oosterveld, Michiel Js, Prikhodina, Larisa, Hoppe, Bernd, Cochat, Pierre and Groothoff, Jaap W. (2022). Long-Term Transplantation Outcomes in Patients With Primary Hyperoxaluria Type 1 Included in the European Hyperoxaluria Consortium (OxalEurope) Registry. Kidney Int. Rep., 7 (2). S. 210 - 221. NEW YORK: ELSEVIER SCIENCE INC. ISSN 2468-0249

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Abstract

Introduction: In primary hyperoxaluria type 1 (PH1), oxalate overproduction frequently causes kidney stones, nephrocalcinosis, and kidney failure. As PH1 is caused by a congenital liver enzyme defect, combined liver-kidney transplantation (CLKT) has been recommended in patients with kidney failure. Nevertheless, systematic analyses on long-term transplantation outcomes are scarce. The merits of a sequential over combined procedure regarding kidney graft survival remain unclear as is the place of isolated kidney transplantation (KT) for patients with vitamin B6-responsive genotypes. Methods: We used the OxalEurope registry for retrospective analyses of patients with PH1 who underwent transplantation. Analyses of crude Kaplan-Meier survival curves and adjusted relative hazards from the Cox proportional hazards model were performed. Results: A total of 267 patients with PH1 underwent transplantation between 1978 and 2019. Data of 244 patients (159 CLKTs, 48 isolated KTs, 37 sequential liver-KTs [SLKTs]) were eligible for comparative analyses. Comparing CLKTs with isolated KTs, adjusted mortality was similar in patients with B6-unresponsive genotypes but lower after isolated KT in patients with B6-responsive genotypes (adjusted hazard ratio 0.07, 95% CI: 0.01-0.75, P = 0.028). CLKT yielded higher adjusted event-free survival and death-censored kidney graft survival in patients with B6-unresponsive genotypes (P = 0.025, P < 0.001) but not in patients with B6-responsive genotypes (P = 0.145, P = 0.421). Outcomes for 159 combined procedures versus 37 sequential procedures were comparable. There were 12 patients who underwent pre- emptive liver transplantation (PLT) with poor outcomes. Conclusion: The CLKT or SLKT remains the preferred transplantation modality in patients with PH1 with B6-unresponsive genotypes, but isolated KT could be an alternative approach in patients with B6-responsive genotypes.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Metry, Elisabeth L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Garrelfs, Sander F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Peters-Sengers, Hessel UNSPECIFIED orcid.org/0000-0003-3145-864X UNSPECIFIED Hulton, Sally-Anne UNSPECIFIED UNSPECIFIED UNSPECIFIED Acquaviva, Cecile UNSPECIFIED UNSPECIFIED UNSPECIFIED Bacchetta, Justine UNSPECIFIED UNSPECIFIED UNSPECIFIED Beck, Bodo B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Collard, Laure UNSPECIFIED UNSPECIFIED UNSPECIFIED Deschenes, Georges UNSPECIFIED UNSPECIFIED UNSPECIFIED Franssen, Casper UNSPECIFIED orcid.org/0000-0003-1004-9994 UNSPECIFIED Kemper, Markus J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lipkin, Graham W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mandrile, Giorgia UNSPECIFIED UNSPECIFIED UNSPECIFIED Mohebbi, Nilufar UNSPECIFIED UNSPECIFIED UNSPECIFIED Moochhala, Shabbir H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Oosterveld, Michiel Js UNSPECIFIED UNSPECIFIED UNSPECIFIED Prikhodina, Larisa UNSPECIFIED UNSPECIFIED UNSPECIFIED Hoppe, Bernd UNSPECIFIED UNSPECIFIED UNSPECIFIED Cochat, Pierre UNSPECIFIED UNSPECIFIED UNSPECIFIED Groothoff, Jaap W. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-696243
DOI:	10.1016/j.ekir.2021.11.006
Journal or Publication Title:	Kidney Int. Rep.
Volume:	7
Number:	2
Page Range:	S. 210 - 221
Date:	2022
Publisher:	ELSEVIER SCIENCE INC
Place of Publication:	NEW YORK
ISSN:	2468-0249
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language LIVER-KIDNEY TRANSPLANTATION; GENOTYPE-PHENOTYPE CORRELATION; SYSTEMIC OXALOSIS; SEQUENTIAL LIVER; AGXT MUTATION; LIVING DONOR; CHILDREN; PYRIDOXINE; DIAGNOSIS; STRATEGY Multiple languages Urology & Nephrology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69624