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SODIUM BENZOATE (BZ) ARRESTS PROGRESSION OF RENAL FAILURE IN INFANTILE PRIMARY OXALURIA (OXU)

Infantile OXU is characterized by oxalate (OX) nephrocalcinosis and rapid progression to end stage renal disease. BZ (which may reduce OX production by diverting its precursors, glycine and glyoxylate, to hippurate synthesis) has not been considered to be useful because short term clinical trials revealed no permanent decrease in OX excretion (BMJ 1:175, '58, AJM 29:820, '60). However, we hypothesize that because of the accumulated body OX burden, high excretion rates may persists even if OX production decreases. The value of BZ may better be judged by longterm outcome. An 8 week old female infant with OXU presented with severe OX nephrocalcinosis, and a serum creatinine (Cr) of 2.5 mg/dl. At 3 m of age her Cr rose to 4.8 and BZ (300 mg/kg/d) was started and continued to the present (25 m) at which time her Cr is 4.8. No OX bone deposition occurred after 8 m of age. Plasma hippurate levels of 4-14 mM produced no adverse affects. Mean plasma BZ level was <0.1 mM. We suggest that BZ reduces OX production, tissue deposition and destruction. Thus BZ may be useful in OXU including such patients who are transplant recipients.