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

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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.

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Nomenclature for kidney function and disease: report of a Kidney Disease: Improving Global Outcomes (KDIGO) Consensus Conference

Andrew S. Levey, Kai-Uwe Eckardt, Nijsje M. Dorman … (2020)

The worldwide burden of kidney disease is rising, but public awareness remains limited, underscoring the need for more effective communication by stakeholders in the kidney health community. Despite this need for clarity, the nomenclature for describing kidney function and disease lacks uniformity. In June 2019, Kidney Disease: Improving Global Outcomes (KDIGO) convened a Consensus Conference with the goal of standardizing and refining the nomenclature used in the English language to describe kidney function and disease, and of developing a glossary that could be used in scientific publications. Guiding principles of the conference were that the revised nomenclature should be patient-centered, precise, and consistent with nomenclature used in the KDIGO guidelines. Conference attendees reached general consensus on the following recommendations: (i) to use "kidney" rather than "renal" or "nephro-" when referring to kidney disease and kidney function; (ii) to use "kidney failure" with appropriate descriptions of presence or absence of symptoms, signs, and treatment, rather than "end-stage kidney disease"; (iii) to use the KDIGO definition and classification of acute kidney diseases and disorders (AKD) and acute kidney injury (AKI), rather than alternative descriptions, to define and classify severity of AKD and AKI; (iv) to use the KDIGO definition and classification of chronic kidney disease (CKD) rather than alternative descriptions to define and classify severity of CKD; and (v) to use specific kidney measures, such as albuminuria or decreased glomerular filtration rate (GFR), rather than "abnormal" or "reduced" kidney function to describe alterations in kidney structure and function. A proposed 5-part glossary contains specific items for which there was general agreement. Conference attendees acknowledged limitations of the recommendations and glossary, but they considered standardization of scientific nomenclature to be essential for improving communication.

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Lumasiran, an RNAi Therapeutic for Primary Hyperoxaluria Type 1

Sander F. Garrelfs, Yaacov Frishberg, Sally Hulton … (2021)

Primary hyperoxaluria type 1 (PH1) is a rare genetic disease caused by hepatic overproduction of oxalate that leads to kidney stones, nephrocalcinosis, kidney failure, and systemic oxalosis. Lumasiran, an investigational RNA interference (RNAi) therapeutic agent, reduces hepatic oxalate production by targeting glycolate oxidase.

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2018 Annual Report of the European Liver Transplant Registry (ELTR) - 50-year evolution of liver transplantation

René Adam, Vincent Karam, Valérie Cailliez … (2018)

The purpose of this registry study was to provide an overview of trends and results of liver transplantation (LT) in Europe from 1968 to 2016. These data on LT were collected prospectively from 169 centers from 32 countries, in the European Liver Transplant Registry (ELTR) beginning in 1968. This overview provides epidemiological data, as well as information on evolution of techniques, and outcomes in LT in Europe over more than five decades; something that cannot be obtained from only a single center experience.

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Author and article information

Contributors

Elisabeth L. Metry

Journal

Journal ID (nlm-ta): Kidney Int Rep

Journal ID (iso-abbrev): Kidney Int Rep

Title: Kidney International Reports

Publisher: Elsevier

ISSN (Electronic): 2468-0249

Publication date PMC-release: 26 November 2021

Publication date Collection: February 2022

Publication date (Electronic): 26 November 2021

Volume: 7

Issue: 2

Pages: 210-220

Affiliations

[1 ]Department of Pediatric Nephrology, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands

[2 ]Center for Experimental and Molecular Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands

[3 ]Department of Nephrology, Birmingham Women’s and Children’s Hospital NHS Foundation Trust, Birmingham, UK

[4 ]Service de Biochimie et Biologie Moléculaire, UM Pathologies Héréditaires du Métabolisme et du Globule Rouge, Hospices Civils de Lyon, France

[5 ]Centre de Référence des Maladies Rares Néphrogones, Hospices Civils de Lyon & Université Claude-Bernard Lyon, Lyon, France

[6 ]Institute of Human Genetics, Center for Molecular Medicine Cologne, University Hospital of Cologne, Cologne, Germany

[7 ]Center for Rare and Hereditary Kidney Disease Cologne, University Hospital of Cologne, Cologne, Germany

[8 ]Department of Pediatric Nephrology, Centre Hospitalier Universitaire Liège, Liège, Belgium

[9 ]Department of Pediatric Nephrology, Assistance Publique–Hôpitaux de Paris Robert-Debré, University of Paris, Paris, France

[10 ]Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

[11 ]Division of Pediatric Nephrology, University Children’s Hospital, Hamburg, Germany

[12 ]Department of Nephrology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

[13 ]Medical Genetics Unit, Department of Clinical and Biological Sciences, University of Torino, Orbassano, Italy

[14 ]Thalassemia Unit, San Luigi University Hospital, Orbassano, Italy

[15 ]Division of Nephrology, University Hospital Zurich, Zurich, Switzerland

[16 ]UCL Department of Renal Medicine, Royal Free Hospital, London, UK

[17 ]Department of Inherited and Acquired Kidney Diseases, Research and Clinical Institute for Pediatrics, Pirogov Russian National Research Medical University, Moscow, Russia

[18 ]Department of Pediatric Nephrology, Children’s Hospital of the University of Bonn, Bonn, Germany

Author notes

[∗ ] 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. e.l.metry@ 123456amsterdamumc.nl

[19]

ELM and SFG are co-first authors.

Article

Publisher Item ID: S2468-0249(21)01512-6

DOI: 10.1016/j.ekir.2021.11.006

PMC ID: 8821040

PubMed ID: 35155860

SO-VID: d3358a40-7fb6-487f-b514-5b9c59798a7a

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 21 September 2021

Date revision received : 28 October 2021

Date accepted : 1 November 2021

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

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Most cited references 38

Nomenclature for kidney function and disease: report of a Kidney Disease: Improving Global Outcomes (KDIGO) Consensus Conference

Lumasiran, an RNAi Therapeutic for Primary Hyperoxaluria Type 1

2018 Annual Report of the European Liver Transplant Registry (ELTR) - 50-year evolution of liver transplantation

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