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.
Subjects: Medicine and Health.