PRIMARY HYPEROXALURIA IN AN ADULT WITH RENAL-FAILURE, LIVEDO-RETICULARIS, RETINOPATHY, AND PERIPHERAL NEUROPATHY

We present the case of a young woman who developed renal failure of un known cause, and after 2 months of maintenance hemodialysis developed livedo reticularis, retinopathy, and peripheral sensory neuropathy. Th e patient was subsequently shown to have primary oxalosis type I, a ra re autosomal recessive error of metabolism characterized by accumulati on of calcium oxalate crystals in the kidneys, eyes, skin, and other o rgans. Intravascular obstruction, caused by deposition of calcium oxal ate crystals in cutaneous arterioles, is thought to be responsible for the ischemic livedo reticularis lesions observed in this patient. A m ethod is described for measuring serum glycolate by isotope dilution g as chromatography-mass spectrometry (GO-MS). An approach to the diagno sis and management is also briefly mentioned. (C) 1997 by the National Kidney Foundation, Inc.