Kidney Transplant Outcomes in Patients With Adenine Phosphoribosyltransferase Deficiency.
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Authors
Runolfsdottir, Hrafnhildur LinnetPalsson, Runolfur
Agustsdottir, Inger M Sch
Indridason, Olafur S
Li, Jennifer
Dao, Myriam
Knebelmann, Bertrand
Milliner, Dawn S
Edvardsson, Vidar O
Issue Date
2020-11
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Runolfsdottir HL, Palsson R, Agustsdottir IMS, Indridason OS, Li J, Dao M, Knebelmann B, Milliner DS, Edvardsson VO. Kidney Transplant Outcomes in Patients With Adenine Phosphoribosyltransferase Deficiency. Transplantation. 2020 Oct;104(10):2120-2128. doi: 10.1097/TP.0000000000003088Abstract
Background: Adenine phosphoribosyltransferase (APRT) deficiency is a rare, hereditary cause of kidney stones and chronic kidney disease (CKD) which is characterized by 2,8-dihydroxyadenine renal parenchymal crystal deposition. The aim of this study was to examine outcomes of kidney transplantation in APRT deficiency patients. Methods: Included were 13 patients in the APRT Deficiency Registry of the Rare Kidney Stone Consortium, 2 from Westmead Hospital in Sydney, Australia, and 2 from Necker Hospital in Paris, France. The CKD-EPI and CKiD equations were used to calculate glomerular filtration rate estimates. Allograft survival was analyzed employing the Kaplan-Meier method. The Wilcoxon-Mann-Whitney test was used to compare alllograft outcomes according to xanthine oxidoreductase (XOR) inhibitor treatment status at transplantation. Results: Seventeen patients (9 females) received 22 kidney transplants. Age at first transplantation was 47.2 (14.9-67.0) years. Ten patients received XOR inhibitor therapy pretransplant (11 allografts), while 8 patients did not receive such treatment before transplantation (11 allografts). Two-year allograft survival was 91% and 55% in the 2 groups, respectively (P = 0.16). The median (range) estimated glomerular filtration rate at 2 years posttransplant was 61.3 (24.0-90.0) mL/min/1.73 m when XOR inhibitor therapy was initiated before transplantation, and 16.2 (10.0-39.0) mL/min/1.73 m (P = 0.009) when such treatment was not administered pretransplant. Conclusions: Kidney allograft outcomes are good in APRT deficiency patients beginning XOR inhibitor therapy pretransplant. Delay in such treatment is a major cause of premature graft loss in these patients. Increased awareness among clinicians is imperative, promoting early diagnosis of APRT deficiency and pharmacotherapy initiation before kidney transplantation.Description
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https://journals.lww.com/transplantjournal/pages/articleviewer.aspx?year=2020&issue=10000&article=00023&type=Fulltextae974a485f413a2113503eed53cd6c53
10.1097/TP.0000000000003088
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