Abstract
We treated 18 children with neonatal onset UCE: CPS 2, OTC 3, AS 6, AL 6. CPS and OTC were treated with protein restriction (PR) 0.5–0.75 g/kg/d + essential amino acids (EAA) 1 g/kg/d + arginine 1 mmol/kg/d + benzoate (B) 1.75 mmol/kg/d. AS was treated with PR + EAA + Arg (3–4 mmol/kg/d) + B. AL was treated with PR + Arg (3–4 mmol/kg/d).
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© 1982 Springer Science+Business Media New York
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Batshaw, M., Brusilow, S. (1982). Therapy of Neonatal Onset Urea Cycle Enzymopathies (UCE). In: Lowenthal, A., Mori, A., Marescau, B. (eds) Urea Cycle Diseases. Advances in Experimental Medicine and Biology, vol 153. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6903-6_16
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DOI: https://doi.org/10.1007/978-1-4757-6903-6_16
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