N-Carbamylglutamate Is an Effective Treatment for Acute Neonatal Hyperammonaemia in a Patient with Methylmalonic Aciduria.

@article{Yap2016NCarbamylglutamateIA,
  title={N-Carbamylglutamate Is an Effective Treatment for Acute Neonatal Hyperammonaemia in a Patient with Methylmalonic Aciduria.},
  author={S. -L. Yap and Huey Yin Leong and Fadzlina Abdul Aziz and Haszlin Hassim and Pavai Sthaneshwar and Ser Huy Teh and Ili Syazwana Abdullah and Lock Hock Ngu and Zulqarnain Mohamed},
  journal={Neonatology},
  year={2016},
  volume={109 4},
  pages={303-7}
}
N-carbamylglutamate (NCG) has been used in combination with ammonia scavengers (sodium benzoate, sodium phenylbutyrate) and dialysis to treat hyperammonaemia in methylmalonic aciduria (MMA). The sole use of NCG for acute neonatal hyperammonaemia secondary to MMA is demonstrated in a neonate presenting at day 9 with encephalopathy, severe metabolic acidosis, hyperammonaemia (1,089 μmol/l), ketonuria and urinary methylmalonic acids. Emergency treatment included discontinuing protein feeds… CONTINUE READING
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