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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