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Published on: July 1, 2012
Primary hyperoxaluria type 1: improved outcome with timely liver transplantation: a single-center report of 36
R Shapiro1, I Weismann, H Mandel
1Pediatric Gastroenterology and Nephrology Units, Schneider Children's Medical Center, Petach-Tikva, 49100, Israel.
Insights
Early liver transplantation improves outcomes for children with type-1 primary hyperoxaluria (PH-1). Timely intervention, including preemptive or combined liver-kidney transplants, is crucial for managing this rare genetic disorder.
Area of Science:
- Pediatric Nephrology and Hepatology
- Genetic Metabolic Disorders
- Organ Transplantation
Background:
- Type-1 primary hyperoxaluria (PH-1) is a rare genetic disorder.
- The optimal management strategy for PH-1 in children, particularly regarding liver transplantation, requires further elucidation.
- This study reviews a decade of experience with PH-1 patients, focusing on liver transplant outcomes.
Purpose of the Study:
- To evaluate the efficacy and outcomes of liver transplantation in children diagnosed with PH-1.
- To determine the appropriate timing and type of liver transplant (preemptive vs. combined liver-kidney) for PH-1 patients.
- To assess the impact of liver transplantation on renal function, oxalate excretion, and overall survival in pediatric PH-1.
Main Methods:
- Retrospective review of 36 children diagnosed with PH-1 from 1989-1998 in northern Israel.
- Analysis of clinical data, including diagnosis, renal failure, transplantation type (kidney-only, combined liver-kidney, preemptive liver), pre- and post-transplant oxalate excretion, renal function (GFR), survival, and complications.
- Comparison of outcomes between transplanted and non-transplanted PH-1 patients.
Main Results:
- Of 12 liver transplant recipients, 10 survived with improved renal function (mean GFR 77 ml/min/m2) and normalized urinary oxalate excretion in 9.
- Combined liver-kidney transplantation (9 patients) and preemptive liver transplantation (3 patients) showed better survival rates compared to non-transplanted patients (14 alive, 9 deceased).
- Isolated liver grafts in recipients with remaining renal oxalate deposits showed less improvement in renal function compared to combined transplant recipients.
Conclusions:
- Early diagnosis and timely liver transplantation are essential for managing PH-1 in children.
- Preemptive isolated liver transplantation is recommended for infants or those with slowly progressive PH-1.
- Combined liver-kidney transplantation is advised for PH-1 patients with established end-stage renal disease.
Background:
The appropriate use of liver transplantation in children with type-1 primary hyperoxaluria (PH-1) is not well established. We reviewed our experience with 36 children with PH-1, including 12 who underwent liver transplantation.
Patients And Methods:
From 1989-1998, 36 children from 10 families in northern Israel were diagnosed with PH-1. Eight children presented with renal failure; seven of these eight had the severe infantile form of the disease. One child was treated with kidney transplantation alone. Combined liver-kidney transplantation has been performed in nine children and preemptive liver transplantation in three children. A review of the patients' charts for the following parameters was performed: age, clinical signs, and renal sonographic findings at diagnosis, age at onset of dialysis, and current status. Type of transplant, pre- and posttransplant urine oxalate excretion, current renal function, survival, and complications were recorded in liver recipients.
Results:
Of the 23 nontransplanted children, 9 died of complications related to severe systemic oxalosis and 14 are alive (mean follow-up, 7.4 years), including 2 who are candidates for transplantation. The child who underwent only kidney transplantation died of unrelated causes. Of the 12 liver recipients, 2 died within the first 3 months posttransplant and another child underwent retransplantation due to hepatic arterial thrombosis. At intervals after transplant ranging from 6-54 months, 10 recipients are alive (7 of the 9 recipients of combined liver-kidney transplants and all 3 recipients of preemptive liver transplants). Mean GFR in the 10 survivors is 77 ml/min/m2. In 9 of these 10, daily urinary oxalate excretion normalized. Renal function has improved (mean GFR 86 vs. 58 ml/min/m2) but renal oxalate deposits remain in the three recipients of isolated liver grafts.
Conclusions:
Our decade-long experience with children with PH-1 supports strategies for early diagnosis and timely liver transplantation. Preemptive isolated liver transplantation should be considered in children who develop the disease during infancy or in those with slowly progressive disease when significant symptoms develop. Combined liver-kidney transplantation is suggested for children with end-stage renal disease.
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