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Abstract: FR-PO749

Combined Liver and Kidney Transplantation from Living Donors in a Young Patient with Primary Hyperoxaluria Type 1: A Case Report

Session Information

Category: Transplantation

  • 2102 Transplantation: Clinical


  • Ahmed, Mohamed Mohyedin, Ain Shams University Faculty of Medicine, Cairo, Egypt
  • ElSharkawy, Magdy, Ain Shams University Faculty of Medicine, Cairo, Egypt
  • Talkhan, Hala, Ain Shams University Faculty of Medicine, Cairo, Egypt
  • Emara, Ahmed, Ain Shams University Faculty of Medicine, Cairo, Egypt

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive inherited disease, caused by mutations in AGXT gene. Combined liver kidney transplant (CLKT) is the preferred transplant option for most patients with PH1 since orthotopic liver transplantation replaces the deficient enzyme, thus restoring normal metabolic oxalate production.

Case Description

We present a case of a 27-year-old male with end-stage kidney disease (ESKD). The patient had a right nephrectomy due to recurrent renal stones and chronic pyelonephritis. Histological analysis of the right kidney revealed calcium oxalate deposition within tubules, and genetic testing confirmed the diagnosis of PH1 with a mutation in the AGXT gene. He was maintained on hemodialysis since 2020. Preoperatively, he patient received daily high flux hemodiafiltration (HDF) sessions for ten days. The CLKT was performed using living donors, with his mother donating a kidney and an unrelated person as a liver donor. Perioperative period was uneventful, with both grafts functioning well. His serum creatinine level returned to normal after three days, and his plasma oxalate level decreased from 65 to 22 µmol/L (normal ≤ 30 µmol/L) after 20 days postoperatively. The patient was maintained on immunosuppressive therapy consisting of prednisolone, mycophenolate mofetil, and tacrolimus after the surgery. The patient maintained stable graft function with no complications during the follow-up period. However, his 24-hour urinary oxalate level remained persistently high despite the normalization of his plasma oxalate level, necessitating the addition of pyridoxine and magnesium citrate to his medication regimen in addition to hyperhydration.


This case report highlights the successful role of CLKT from living donors in a patient with PH1 with good outcome. Also, preoperative HDF dialysis intensification reduced the oxalate burden, and may reduce incidence of oxalate deposition postoperatively. Crystallization inhibitors may be used postoperatively for a long time to prevent urinary oxalate deposition.