Fig. 1.

Treatment and clinical course over time.

The first onset of hyperammonemia was identified on day 2. Ammonia and plasma glutamine levels decreased immediately after initiation of acute therapy and remained within the normal range. As her ammonia levels slightly increased due to hypercatabolism, we initiated total parenteral nutrition. We started enteral alimentation on day 8, and gradually increased protein intake until she was discharged from the hospital. Due to excessive excretion of glutamine, we reduced the dose of sodium phenylbutyrate and sodium benzoate. The dose of NCG was maintained at a constant level relative to the patient's body weight. We restarted sodium benzoate treatment when her glutamine levels increased again. She remained metabolically stable and did not experience a metabolic crisis throughout her treatment.

NCG, N-carbamylglutamate; CHDF, continuous hemodiafiltration.