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argininosuccinic aciduria

argininosuccinic aciduria A genetic disease affecting the formation of urea as the end‐product of protein metabolism. Depending on the severity of the condition, affected infants may become comatose and die after a moderately high intake of protein. Treatment is by restriction of protein intake and feeding supplements of the amino acid arginine, which permits elimination of nitrogenous waste as argininosuccinic acid. Sodium benzoate may be given to increase the excretion of nitrogenous waste as hippuric acid. See also benzoic acid.

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argininaemia

argininaemia A genetic disease affecting the metabolism of the amino acid arginine, and hence the normal formation of urea as the end‐products of protein metabolism. Depending on the severity of the condition, affected infants may become comatose and die after a moderately high intake of protein. Treatment is by severe restriction of protein intake. Sodium benzoate may be given to increase the excretion of nitrogenous waste as hippuric acid. See also benzoic acid.

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sodium benzoate

sodium benzoate or benzoate of soda, chemical compound, C6H5CO2Na, colorless or white crystalline, aromatic compound; the sodium salt of benzoic acid. It is soluble in water and is used as a preservative in foods and beverages; because it is poisonous, the concentration is limited by law to 0.1%.

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