原發性高草酸尿症循序肝腎移植術後移植腎衰竭:病例報告與文獻回顧

管乙融 陳穎政 高建璋

國防醫學大學三軍總醫院外科部泌尿外科

Kidney Allograft Failure Following Sequential Liver–Kidney Transplantation in a Patient with Primary Hyperoxaluria: A Case Report and Literature Review

Yi-Rong Guan, Ying-Zheng Chen, Chien-Chang Kao

Division of Urology, Department of Surgery, Tri-Service General Hospital, National Defense Medical University

Abstract: Primary hyperoxaluria type 1 is a devasting autosomal recessive disorder with mutations in AGXT, the gene encoding AGT, involving the overproduction of oxalate leading to systemic oxalosis which encompass various systems, including integumentary, skeletal, digestive, cardiovascular, ophthalmologic, hematologic, urinary, and the central nervous system. Liver–kidney transplantation remains the only curative therapy for end-stage renal disease (ESRD) in PH1. We report a case of kidney allograft failure following sequential liver–kidney transplantation in a patient with PH1 and provide a review of current literature.

 A 27-year-old male with genetically confirmed primary hyperoxaluria type 1 (PH1) and systemic oxalosis involving the kidneys, bone marrow, and tonsils was maintained on regular hemodialysis following the progression to end-stage renal disease (ESRD). He underwent living donor liver transplantation from his mother on October 3, 2022 (2022-10-03) to correct the underlying metabolic defect. Postoperatively, his plasma oxalate levels decreased significantly, reaching levels below 5
μmol/L, indicating a biochemical response to liver transplantation. Subsequently, on May 5, 2025 (2025-05-05), he received a deceased donor kidney. transplant. Despite initially adequate graft perfusion, the patient developed early allograft dysfunction within two weeks post-transplant. Renal biopsy revealed extensive oxalate crystal deposition with associated tubular injury, confirming oxalate nephropathy as the cause of allograft failure. The patient resumed hemodialysis thereafter.