Long-Term Transplantation Outcomes in Patients With Primary Hyperoxaluria Type 1 Included in the European Hyperoxaluria Consortium (OxalEurope) Registry

Elisabeth L. Metry; Sander F. Garrelfs; Hessel Peters-Sengers; Sally-Anne Hulton; Cecile Acquaviva; Justine Bacchetta; Bodo B. Beck; Laure Collard; Georges Deschênes; Casper Franssen; Markus J. Kemper; Graham W. Lipkin; Giorgia Mandrile; Nilufar Mohebbi; Shabbir H. Moochhala; Michiel J.S. Oosterveld; Larisa Prikhodina; Bernd Hoppe; Pierre Cochat; Jaap W. Groothoff

Kidney International Reports (Feb 2022)

Long-Term Transplantation Outcomes in Patients With Primary Hyperoxaluria Type 1 Included in the European Hyperoxaluria Consortium (OxalEurope) Registry

Elisabeth L. Metry,
Sander F. Garrelfs,
Hessel Peters-Sengers,
Sally-Anne Hulton,
Cecile Acquaviva,
Justine Bacchetta,
Bodo B. Beck,
Laure Collard,
Georges Deschênes,
Casper Franssen,
Markus J. Kemper,
Graham W. Lipkin,
Giorgia Mandrile,
Nilufar Mohebbi,
Shabbir H. Moochhala,
Michiel J.S. Oosterveld,
Larisa Prikhodina,
Bernd Hoppe,
Pierre Cochat,
Jaap W. Groothoff

Affiliations

Elisabeth L. Metry: Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; Correspondence: Elisabeth L. Metry, Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Room H8-253, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Sander F. Garrelfs: Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
Hessel Peters-Sengers: Center for Experimental and Molecular Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
Sally-Anne Hulton: Department of Nephrology, Birmingham Women’s and Children’s Hospital NHS Foundation Trust, Birmingham, UK
Cecile Acquaviva: Service de Biochimie et Biologie Moléculaire, UM Pathologies Héréditaires du Métabolisme et du Globule Rouge, Hospices Civils de Lyon, France
Justine Bacchetta: Centre de Référence des Maladies Rares Néphrogones, Hospices Civils de Lyon & Université Claude-Bernard Lyon, Lyon, France
Bodo B. Beck: Institute of Human Genetics, Center for Molecular Medicine Cologne, University Hospital of Cologne, Cologne, Germany; Center for Rare and Hereditary Kidney Disease Cologne, University Hospital of Cologne, Cologne, Germany
Laure Collard: Department of Pediatric Nephrology, Centre Hospitalier Universitaire Liège, Liège, Belgium
Georges Deschênes: Department of Pediatric Nephrology, Assistance Publique–Hôpitaux de Paris Robert-Debré, University of Paris, Paris, France
Casper Franssen: Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
Markus J. Kemper: Division of Pediatric Nephrology, University Children’s Hospital, Hamburg, Germany
Graham W. Lipkin: Department of Nephrology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Giorgia Mandrile: Medical Genetics Unit, Department of Clinical and Biological Sciences, University of Torino, Orbassano, Italy; Thalassemia Unit, San Luigi University Hospital, Orbassano, Italy
Nilufar Mohebbi: Division of Nephrology, University Hospital Zurich, Zurich, Switzerland
Shabbir H. Moochhala: UCL Department of Renal Medicine, Royal Free Hospital, London, UK
Michiel J.S. Oosterveld: Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
Larisa Prikhodina: Department of Inherited and Acquired Kidney Diseases, Research and Clinical Institute for Pediatrics, Pirogov Russian National Research Medical University, Moscow, Russia
Bernd Hoppe: Department of Pediatric Nephrology, Children’s Hospital of the University of Bonn, Bonn, Germany
Pierre Cochat: Centre de Référence des Maladies Rares Néphrogones, Hospices Civils de Lyon & Université Claude-Bernard Lyon, Lyon, France
Jaap W. Groothoff: Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands

Journal volume & issue: Vol. 7, no. 2
pp. 210 – 220

Abstract

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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.

Published in Kidney International Reports

ISSN: 2468-0249 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the genitourinary system. Urology
Website: http://www.journals.elsevier.com/kidney-international-reports/

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