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Table 1 Analysis of plasma amino acids and urinary orotic acid

From: Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting

  CPS-1 deficiency OTC deficiency ASS-1 deficiency ASL deficiency
Citrulline Absent Low Elevated (tenfold) Elevated (twofold)
Arginine Reduced Reduced Reduced Reduced
Orotic acid Low Elevated Elevated Elevated