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This Concept Map, created with IHMC CmapTools, has information related to: Biochem A1 Error of Urea Synthesis, Sodium Phenylacetate conjugated with glutamine, elevated glutamine levels prompting genetic testing, Oxaloacetate decreased levels of Glucose, Blood glucose levels reduces dependence on protein, NH3 carried to the liver by alanine, protein used to reduce NH3, glutamine producing phenylacetylglutamine, urine reducing elevated glutamine levels, Oxaloacetate form alpha ketoglutarate, carbamoyl phosphate synthetase I gene which normally encodes for carbomoyl phosphate synthetase I, ornithine transporter 1 where it reacts with ornithine transcarbamoylase, urea cycle leading to build up NH3, Citrulline transported to cytosol, undetectable citrulline prompting genetic testing, arginase producing urea, respiratory alkalosis prompting genetic testing, Argininosuccinate lyase to form arginine, L-arginine infusion provides pt with arginine, Infant Patient presents with mild respiratory distress, testing showing elevated glutamine levels