Therapy of urea cycle enzymopathies: Three case studies

M. L. Batshaw, M. J. Painter, G. T. Sproul, I. A. Schafer, G. H. Thomas, S. Brusilow

Research output: Contribution to journalArticlepeer-review

21 Scopus citations


Complete inactivity of any of the urea cycle enzymes (other than arginase) leads to the accumulation of ammonium. We recently suggested treatment of defects of the urea cycle by employing alternative pathways of waste nitrogen excretion. We report the long-term therapy of three infants with congenital urea cycle enzymopathies using a combination of protein restriction and dietary supplementation of essential amino acids, arginine, and sodium benzoate.

Original languageEnglish (US)
Pages (from-to)34-40
Number of pages7
JournalJohns Hopkins Medical Journal
Issue number1
StatePublished - 1981

ASJC Scopus subject areas

  • General Medicine


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