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Published on: September 13, 2014
Liver Transplantation in Primary Hyperoxaluria: A Single-Center 10-Year Experience
Zahra Beyzaei1, Alireza Shamsaeefar2, Kiana Ghatei1
1Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Pediatric Transplantation
|May 21, 2025
Summary
Liver transplantation (LT) significantly improves outcomes for patients with primary hyperoxalurias (PHs), a rare metabolic disorder. Post-transplant, patients experienced normalized liver enzymes and reduced urine oxalate levels, enhancing survival and quality of life.
Area of Science:
- Metabolic Disorders
- Organ Transplantation
- Nephrology
Background:
- Primary hyperoxalurias (PHs) are rare genetic metabolic disorders characterized by excessive oxalate production and deposition.
- Untreated PHs can lead to severe complications including end-stage kidney disease (ESKD) and mortality.
- Liver transplantation (LT) is an established treatment for PHs, particularly in pediatric cases.
Purpose of the Study:
- To evaluate the long-term outcomes of liver transplantation (LT) in patients with primary hyperoxalurias (PHs).
- To analyze post-transplantation results, including survival rates and biochemical markers, in a single-center cohort in Iran.
Main Methods:
- Retrospective analysis of 33 liver transplant recipients with PHs from 2012 to 2023.
- Evaluation of both deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT).
- Assessment of biochemical data, perioperative factors, and survival using Kaplan-Meier analysis.
Main Results:
- All 33 patients showed normalized liver enzymes post-LT, with a significant decrease in urine oxalate levels.
- Patient survival rates at 5 years were 85%, with graft survival at 97%.
- Eight patients experienced acute rejection, and five had chronic rejection; 21 patients required prior or concurrent kidney transplantation.
Conclusions:
- Liver transplantation (LT) offers significant survival and quality of life benefits for patients with primary hyperoxalurias (PHs).
- The study demonstrated favorable long-term outcomes and reduced oxalate levels post-LT, but challenges like graft availability and renal graft loss persist.
- Close monitoring and multidisciplinary care are crucial for managing PH patients post-transplantation.
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