ESPN 53rd Annual Meeting

ESPN 2021


 
Long-term transplantation outcomes in patients with primary hyperoxaluria type I: results from the OxalEurope database
ELISABETH METRY 1 SANDER GARRELFS 1 HESSEL PETERS-SENGERS 2 MICHIEL OOSTERVELD 1 GILL RUMSBY 3 GRAHAM LIPKIN 4 SALLY-ANN HULTON 5 SHABBIR MOOCHHALLA 6 JUSTINE BACCHETTA 7 GEORGES DESCHENES 8 CECILE ACQUAVIVA 9 BODO BECK 10 BERND HOPPE 11 MARKUS KEMPER 12 CASPER FRANSSEN 13 GEORGIA MANDRILE 14 LAURE COLLARD 15 NILUFAR MOHEBBI 16 LARISA PRIKHODINA 17 JAAP GROOTHOFF 1

1- DEPARTMENT OF PEDIATRIC NEPHROLOGY, EMMA CHILDRENS 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 CLINICAL BIOCHEMISTRY, UNIVERSITY COLLEGE LONDON HOSPITALS, LONDON, UK.
4- DEPARTMENT OF NEPHROLOGY, UNIVERSITY HOSPITALS BIRMINGHAM NHS FOUNDATION TRUST, BIRMINGHAM, UK.
5- DEPARTMENT OF NEPHROLOGY, BIRMINGHAM CHILDRENS HOSPITAL NHS FOUNDATION TRUST, BIRMINGHAM, UK.
6- KIDNEY AND UROLOGY CENTRE, ROYAL FREE HOSPITAL, LONDON, UK.
7- DEPARTMENT OF PEDIATRIC NEPHROLOGY, RHEUMATOLOGY AND DERMATOLOGY, UNIVERSITY CHILDRENS HOSPITAL, LYON, FRANCE.
8- DEPARTMENT OF PEDIATRIC NEPHROLOGY, ASSISTANCE PUBLIQUE-HôPITAUX DE PARIS, HôPITAL ROBERT DEBRé, UNIVERSITY OF PARIS, PARIS, FRANCE.
9- FéDéRATION DE GéNéTIQUE, ASSISTANCE PUBLIQUE-HôPITAUX DE PARIS, HôPITAL ROBERT DEBRé, UNIVERSITY OF PARIS, PARIS, FRANCE.
10- INSTITUTE OF HUMAN GENETICS, CENTER FOR MOLECULAR MEDICINE COLOGNE, AND CENTER FOR RARE AND HEREDITARY KIDNEY DISEASE, UNIVERSITY HOSPITAL OF COLOGNE, COLOGNE, GERMANY.
11- DEPARTMENT OF PEDIATRIC NEPHROLOGY, CHILDRENS HOSPITAL OF THE UNIVERSITY OF BONN, BONN, GERMANY.
12- DIVISION OF PEDIATRIC NEPHROLOGY, UNIVERSITY CHILDRENS HOSPITAL, HAMBURG, GERMANY.
13- DEPARTMENT OF INTERNAL MEDICINE, UNIVERSITY MEDICAL CENTER GRONINGEN, UNIVERSITY OF GRONINGEN, GRONINGEN, THE NETHERLANDS
14- MEDICAL GENETICS UNIT, DEPARTMENT OF CLINICAL AND BIOLOGICAL SCIENCES, UNIVERSITY OF TORINO, ORBASSANO (TO), ITALY; GENETICA E THALASSEMIA UNIT, SAN LUIGI UNIVERSITY HOSPITAL, ORBASSANO (TO), ITALY.
15- DEPARTMENT OF PEDIATRIC NEPHROLOGY, CHU LIèGE, LIèGE, BELGIUM.
16- DIVISION OF NEPHROLOGY, UNIVERSITY HOSPITAL ZURICH, ZURICH, SWITZERLAND
17- DEPARTMENT OF INHERITED AND ACQUIRED KIDNEY DISEASES, RESEARCH AND CLINICAL INSTITUTE FOR PEDIATRICS, PIROGOV RUSSIAN NATIONAL RESEARCH MEDICAL UNIVERSITY, MOSCOW, RUSSIA.
 
Introduction:

 

Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder causing calcium-oxalate kidney stones and often kidney failure. Since this is due to overproduction of oxalate by the liver, combined liver-kidney transplantation (CLKT) is recommended. However, the advantage of a sequential procedure (SLKT) remains unclear as well as the place of isolated kidney transplant (KT) for patients in whom vitamin B6 lowers hepatic oxalate production effectively. Data on long-term transplantation outcomes are scarce.

Material and methods:

 

We retrospectively analyzed data from the OxalEurope registry. Out of approximately 1100 PH patients, we selected PH1 patients who underwent one or more transplantations. Survival analyses were performed using Kaplan Meier method. 

Results:

 

In total, 267 PH1 patients underwent transplantation between 1978 and 2019. Data of 244  transplantations were eligible for comparative analyses. Median (interquartile range) follow-up was 6.0 years (1.9-11.5). Event-free survival was better for CLKT compared to KT in B6 unresponsive patients (p<0.001), but not for CLKT compared to KT in B6 responsive patients (p=0.411), while death-censored kidney graft survival was superior for CLKT in both groups. Comparable outcomes were found for CLKT (n=159) and SLKT (n=37). Twelve patients underwent pre-emptive liver transplantations with poor outcomes.

Conclusions:

 

Combined or sequential liver-kidney transplantation remains the preferred transplantation modality in B6 unresponsive patients with kidney failure. Our findings support the choice for isolated KT in B6 responsive patients.