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Therapy of Neonatal Onset Urea Cycle Enzymopathies (UCE)

  • M. Batshaw
  • S. Brusilow
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

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 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • M. Batshaw
    • 1
  • S. Brusilow
    • 1
  1. 1.Johns Hopkins Medical InstituteBaltimoreUSA

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