A 6-month-old male infant presents with poor feeding, lethargy, and vomiting after starting protein-rich feeds. Plasma ammonia is 180 µmol/L (normal <50). Genetic testing reveals a deficiency in carbamoyl phosphate synthetase I (CPS I). Which clinical and biochemical feature best distinguishes CPS I deficiency from ornithine transcarbamylase (OTC) deficiency?

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