Advertisement

Guanidinosuccinic Acid Excretion in Argininosuccinic Aciduria

  • H. Böhles
  • B. D. Cohen
  • D. Michalk
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

Guanidinosuccinic acid (GSA) is a normally occurring metabolite in serum and urine of human beings. An intact pathway of urea formation seems to be necessary for its formation because from different inborn defects of urea synthesis the excretion of only minute amounts of GSA is reported (Stein et al. 1969). States of nitrogen retention, especially renal failure, show increased concentrations of GSA in serum and urine (Stein et al. 1969). We want to present our data on the urinary GSA excretion in a patient with argininosuccinic aciduria during the first two weeks after the introduction of ketoanalogues of essential amino acids into the dietary regime.

Keywords

Essential Amino Acid Branch Chain Amino Acid Inborn Error Urea Cycle Plasma Amino Acid 
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.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Colombo, J.P., Bachman, C., Terheggen, H.G., Lavinha, F., Lowenthal, A., 1976, Argininemia, in: “The Urea Cycle”, S. Grisolia, R. Baguena, F. Mayor, eds., John Wiley Sons, New York.Google Scholar
  2. Dobbelstein, H., Edel, H.H., Schmidt, M., Schubert, G., Weinzierl, M., 1971, Guanidinobernsteinsäure und Urämie, Klin. W’schr. 49: 348.PubMedCrossRefGoogle Scholar
  3. Kopple, J.D., Gordon, S., Wang, M., 1971, Effect of chronic uremia protein intake and hemodialysis on guanidinosuccinic acid levels, Abstracts of Am. Soc. Nephrol. 5: 41.Google Scholar
  4. Menyhart, J., Grof, J., Somogyi, J. Abstracts, 9th Ann. Meeting Soc. Clin. Invest. Rotterdam 1975.Google Scholar
  5. Stein, J.M., Cohen, B.D., Kornhauser, R.S. 1969, Guanidinosuccinic acid in renal failure, experimental azotemia and inborn errors of the urea cycle, New Engl. J. Med. 280: 926.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • H. Böhles
    • 1
  • B. D. Cohen
    • 2
  • D. Michalk
    • 1
  1. 1.UniversitätskinderklinikErlangenF.R. Germany
  2. 2.Bronx Lebanon HospitalNew YorkUSA

Personalised recommendations