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 language | English (US) |
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Pages (from-to) | 881-887 |
Number of pages | 7 |
Journal | Pediatric Nephrology |
Volume | 33 |
Issue number | 5 |
DOIs | |
State | Published - May 1 2018 |
Externally published | Yes |
Keywords
- Hyperoxaluria
- Kidney transplant
- Liver transplant
- Liver–kidney transplant
- Oxalate
- Pediatric
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Nephrology