Abstract
Recent reports indicate that acylation of glycine by Sodium Benzoate (SB) could be a useful mechanism for the excretion of nitrogen overload as hippurate in congenital hyperammonemias. We have treated a 16 year old French-Canadian girl, confirmed homozygote for hyperargininemia, by a 4 week SB therapy trial.During the control period (0.5g protein/kg BW diet), her fasting plasma NH3N was 130-273 μg/dl (normal 50 ± 13), glutamine:763-776 μg/dl (normal: 369-660) and urinary orotate: 62-516 mg/d (normal: 3 ± 1). Due to feeding difficulties,continuous intragastric infusion was started (1600 Kcal, 0.5g protein/kg), which stabilised NH3N at 93-107, glutamine at 503-754 and orotate at 5-12.
SB (250 mg/kg BW/d), added to infusion, brought NH3N down to 75-88, glutamine to 269-418 and orotate to < 3mg. An attempt to increase the protein intake to 0.8 g/kg was not successful, as the patient gradually developed vomiting and a hyperammonemic crisis. However, reduction of protein intake (0.25 - 0.37 g/kg) and increase in SB (333 mg/kg/d) quickly controlled the crisis. The girl was discharged after 10-day stable period on 0.5 g protein/kg BW mixed diet with oral SB. During this period her NH3N remained at 32-48, glutamine at 252-434 and orotate at < 3 mg/d.
Measurement of urinary hippurate and partition of urinary N shows that SB therapy increases waste N excretion in hyperargininemia. On equal protein intake, hippurate N excretion increased from 2% of total N output without SB to 27% with SB.
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Qureshi, I., Letarte, J., Ouellet, R. et al. 1174 SODIUM BENZOATE THERAPY AND DIETARY CONTROL IN HYPERARGININEMIA. Pediatr Res 15 (Suppl 4), 638 (1981). https://doi.org/10.1203/00006450-198104001-01200
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DOI: https://doi.org/10.1203/00006450-198104001-01200
