Bilateral native nephrectomy to reduce oxalate stores in children at the time of combined liver–kidney transplantation for primary hyperoxaluria type 1

Eliza Lee, Gabriel Ramos-Gonzalez, Nancy Rodig, Scott Elisofon, Khashayar Vakili, Heung Bae Kim

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: Primary hyperoxaluria type-1 (PH-1) is a rare genetic disorder in which normal hepatic metabolism of glyoxylate is disrupted resulting in diffuse oxalate deposition and end-stage renal disease (ESRD). While most centers agree that combined liver–kidney transplant (CLKT) is the appropriate treatment for PH-1, perioperative strategies for minimizing recurrent oxalate-related injury to the transplanted kidney remain unclear. We present our management of children with PH-1 and ESRD on hemodialysis (HD) who underwent CLKT at our institution from 2005 to 2015. Methods: On chart review, three patients (2 girls, 1 boy) met study criteria. Two patients received deceased-donor split-liver grafts, while one patient received a whole liver graft. All patients underwent bilateral native nephrectomy at transplant to minimize the total body oxalate load. Median preoperative serum oxalate was 72 μmol/L (range 17.8–100). All patients received HD postoperatively until predialysis serum oxalate levels fell <20 μmol/L. All patients, at a median of 7.5 years of follow-up (range 6.5–8.9), demonstrated stable liver and kidney function. Conclusions: While CLKT remains the definitive treatment for PH-1, bilateral native nephrectomy at the time of transplant reduces postoperative oxalate stores and may mitigate damage to the renal allograft.

Original languageEnglish (US)
Pages (from-to)881-887
Number of pages7
JournalPediatric Nephrology
Volume33
Issue number5
DOIs
StatePublished - May 1 2018
Externally publishedYes

Keywords

  • Hyperoxaluria
  • Kidney transplant
  • Liver transplant
  • Liver–kidney transplant
  • Oxalate
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

Fingerprint Dive into the research topics of 'Bilateral native nephrectomy to reduce oxalate stores in children at the time of combined liver–kidney transplantation for primary hyperoxaluria type 1'. Together they form a unique fingerprint.

Cite this