Urea Cycle Diseases pp 127-127 | Cite as
Therapy of Neonatal Onset Urea Cycle Enzymopathies (UCE)
Chapter
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).
Keywords
Weight Gain Plasma Level Linear Growth Essential Amino Acid Transient Increase
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Copyright information
© Springer Science+Business Media New York 1982