Abstract: TH-PO766
The Secret Ingredient for Isolated Kidney Transplantation with Primary Hyperoxaluria: A Case Report
Session Information
- Transplantation: Clinical - 1
October 24, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Hryzak, Sarah, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Showers, Christopher R., Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Ahmad, Zahra, University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Atlas, Olivia, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Hummel, Katie, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Abu Gazala, Samir, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Hussain, Sabiha Malik, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
Introduction
Primary hyperoxaluria is defined by a group of autosomal recessive conditions that cause alteration of oxalate metabolism leading to complications such as kidney failure. Simultaneous liver-kidney transplantation (SLKT) has been the treatment of choice for primary hyperoxaluria however newer therapies with RNA interference (RNAi) may offer alternative options. Here we outline both the utilization of RNAi therapy as well as specifics of perioperative kidney replacement therapies for successful isolated kidney transplant (KT).
Case Description
25-year-old with hypertension diagnosed with primary hyperoxaluria at nine months old experienced multiple kidney stones and eventually progressed to advanced kidney disease. She was treated with pyridoxine with reduction in nephrolithiasis. She then received Lumasiran injections for 23 months prior to receiving KT. Plasma oxalate levels were monitored and a level of ≤20umol/L was targeted prior to KT. To achieve this, one week prior to KT, patient underwent daily four-hour hemodialysis sessions for five consecutive days to optimize oxalate clearance. She then underwent isolated living-unrelated KT with immediate graft function followed by three consecutive days of hemodialysis (Figure1). Patient was induced with thymoglobulin and maintained on tacrolimus, mycophenolate and prednisone. She required no further treatments and continues to have excellent allograft function with creatinine of 1.2mg/dL seven months post KT.
Discussion
Primary hyperoxaluria is a devastating disease that for decades has been treated with SLKT. This case highlights the ability to avoid dual organ transplantation and provides granular details of perioperative renal replacement management utilized for a successful KT. By continuing to expand our knowledge on treatment for primary hyperoxaluria, we can reduce risks associated with multi-organ transplantation as well as increase organ availability worldwide.