698 Therapy of Neonatal Onset Urea Cycle Enzymopathies, (uce)


We treated 12 children with neonatal onset UCE: CPS 1, OTC 3, AS 4, AL 4. CPS and OTC were treated with protein restriction (PR) 0.5-lg/kg/d + essential amino acids (EAA) 1g/kg/d + arginine lmmol/kg/d + benzoate (B) 1.75 mmol/kg/d. AS was treated with PR + EAA + Arg (3-4mmol/kg/d) + B. AL was treated with PR + Arg (3-4mmol/kg/d). All patients are alive… (More)
DOI: 10.1203/00006450-198104001-00721


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