End-Stage Renal Failure in Primary Hyperoxaluria Type 2

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Abstract
Primary hyperoxaluria with l-glyceric aciduria (primary hyperoxaluria type 2) is caused by a deficiency of d-glycerate dehydrogenase,1 which catalyzes the conversion of hydroxypyruvate to d-glycerate. Because this enzyme also has glyoxylate reductase activity, glyoxylate accumulates. In the type 1 variant, glyoxylate accumulation is caused by a deficiency of alanine-glyoxylate aminotransferase, which catalyzes the transamination of glyoxylate and alanine to glycine and pyruvate2. Because glyoxylate is the major precursor of oxalate, both types lead to oxalate overproduction and increased urinary oxalate excretion. Despite these similarities, type 1 hyperoxaluria commonly causes end-stage renal failure and systemic oxalosis, whereas for unknown reasons, type 2 hyperoxaluria rarely does so.