Liver-kidney transplantation in primary hyperoxaluria type-1: case report and literature review.

[primary hyperoxaluria type 1]

Primary hyperoxaluria type -1 (PH 1 ) is a rare inherited autosomal recessive disorder in which a deficiency of the hepatic enzyme alanine-glyoxylate aminotransferase leads to endogenous oxalate overproduction , renal failure , systemic oxalate deposition and death . As hemodialysis provides insufficient oxalate clearance , patients ultimately require both liver and kidney transplantation for correction of the metabolic abnormality and oxalate excretion . Herein , we describe a young adult male with end-stage renal disease and systemic oxalosis causing progressive disabling multi-organ dysfunction while awaiting transplantation . We review the literature regarding liver -kidney transplantation and suggest that for patients with PH 1 , a standardized assessment of organ dysfunction and functional impairment may improve identification of patients requiring urgent transplantation thereby reducing the morbidity and mortality that can occur with delayed transplantation .