Abstract: SA-PO1218
Transplant Decision-Making in Primary Hyperoxaluria Type 1 in the Era of Lumasiran
Session Information
- Transplantation: Clinical - Complications, Pediatrics, and Multi-Organ Considerations
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Ojha, Vishnu Shankar, Mayo Clinic in Florida, Jacksonville, Florida, United States
- Wadei, Hani, Mayo Clinic in Florida, Jacksonville, Florida, United States
- Keddis, Mira T., Mayo Clinic Arizona, Scottsdale, Arizona, United States
- Baker, Lyle Wesley, Mayo Clinic in Florida, Jacksonville, Florida, United States
- Porter, Ivan E., Mayo Clinic in Florida, Jacksonville, Florida, United States
Introduction
The emergence of lumasiran has reshaped the management landscape of Primary Hyperoxaluria type 1 (PH1) and introduced nuanced approaches in transplant decision-making, particularly in patients with ESKD and high systemic oxalate burden. We present a case highlighting an individualized approach for transplantation with lumasiran therapy.
Case Description
A 33-year-old woman was referred for kidney transplant evaluation after developing ESKD of unclear etiology requiring maintenance hemodialysis thrice a week. Imaging revealed bilateral cortical nephrocalcinosis and multiple non-obstructing renal calculi. Her past history was notable for recurrent nephrolithiasis. Secondary causes of hyperoxaluria were excluded. Biochemical evaluations are mentioned in Table 1. Genetic testing identified a pathogenic AGXT mutation consistent with PH1. Lumasiran therapy was started. The multidisciplinary team selected a staged liver–kidney transplantation strategy. The patient underwent orthotopic liver transplantation with intraoperative continuous renal replacement therapy. Postoperatively, she remained on maintenance hemodialysis while awaiting kidney transplantation.
Discussion
This case highlights critical aspects in the contemporary management of PH1: delayed diagnosis in adults, the evolving role of lumasiran therapy in ESKD, and the importance of transplant sequencing in the setting of systemic oxalate burden. While lumasiran substantially reduces hepatic oxalate production and may support isolated kidney transplantation in patients with mild PH1, its role in patients with systemic oxalosis remains limited. Our case illustrates the rationale for pursuing definitive liver transplantation despite biochemical improvement with lumasiran, which was used as bridging therapy given the high systemic oxalate burden, dialysis dependence, and risk of recurrent graft oxalosis.