Joint Liver-Kidney Transplantation for Primary Hyperoxaluria and Other Metabolic Conditions

Abstract

Combined liver-kidney transplantation (CLKT) represents a pivotal therapeutic advancement in the management of metabolic disorders, particularly primary hyperoxaluria type I (PH-I), a rare autosomal recessive condition resulting in systemic oxalosis and end-stage renal disease (ESRD). This review synthesizes clinical evidence from key studies, including the University of Kansas Medical Center and international registries, to assess the efficacy, outcomes, and current management strategies associated with CLKT in PH-I and other metabolic diseases. CLKT effectively corrects the underlying hepatic enzymatic deficiency and restores renal function, significantly improving patient and graft survival rates compared to kidney-alone transplantation, which is often compromised by recurrent oxalate deposition. The review outlines perioperative management strategies such as intensive dialysis, high-volume diuresis, and pyridoxine therapy, and highlights critical considerations like timing of transplantation, oxalate mobilization, and immunosuppressive regimens. Despite ongoing challenges, including persistent post-transplant hyperoxaluria and risk of graft rejection, CLKT remains the gold standard for pyridoxine-unresponsive PH-I. Emerging approaches such as gene therapy, improved diagnostic tools, and personalized treatment protocols offer promising avenues for future care.