Leucine turnover in maple syrup urine disease

  • J. V. Leonard
  • A. M. Umpleby
  • E. M. Naughten
  • M. A. Boroujerdi
  • P. H. Sonksen
Short Communication

Keywords

Public Health Internal Medicine Leucine Metabolic Disease Maple Syrup Urine Disease 

References

  1. Blackburn, G. L., Desai, S. P., Keenan, R. A., Bentley, B. T., Moldawer, L. L. and Bistrian, B. R. Clinical use of BCAA enriched solutions in the stressed and injured patient. In Walser, M. and Williamson, J. R. (eds.)Metabolism and Clinical Implications of Branched Chain Amino Acids and Ketoacids. Elsevier North Holland, Amsterdam, 1982, pp. 521–526Google Scholar
  2. Clow, C., Reade, T. M. and Scriver, C. R. Outcome of early and long term management of classical maple syrup urine disease.Pediatrics 68 (1981) 856–862PubMedGoogle Scholar
  3. Dancis, J. and Levitz, M. Abnormalities of branched chain amino acid metabolism. In Stanbury, J. B., Wyngaarden, J. B. and Fredrickson, D. S. (eds.)The Metabolic Basis of Inherited Disease, 4th Ed. McGraw Hill, New York, 1978, pp. 397–410Google Scholar
  4. Saunders, J., Boroujerdi, M. A., Brown, P. M., Carsons, E. R., Hall, S. E. H., Umpleby, A. M. and Sonksen, P. H. Isotope studies in uncontrolled diabetes and the effects of insulin. InMetabolic Acidosis. Ciba Foundation Symposium 87. Pitman London, 1982, pp. 273–284Google Scholar
  5. Umpleby, A. M., Boroujerdi, M. A., Brown, P. M., Carson, E. R. and Sonksen, P. H. A compartment model of leucine and bicarbonate metabolism in man.Clin. Sci. 61 (1981) 45PGoogle Scholar
  6. Wendel, U., Lombeck, I., Bremer, H. J. and Langenbeck, V. Insulin to assist treatment of acute episodes in maple syrup urine disease.J. Inher. Metab. Dis. 5 Suppl. 1 (1982) 25–26Google Scholar

Copyright information

© SSIEM and MTP Press Limited 1983

Authors and Affiliations

  • J. V. Leonard
    • 1
    • 2
  • A. M. Umpleby
    • 1
    • 2
  • E. M. Naughten
    • 1
    • 2
  • M. A. Boroujerdi
    • 1
    • 2
  • P. H. Sonksen
    • 1
    • 2
  1. 1.Institute of Child HealthLondonUK
  2. 2.the Department of MedicineSt. Thomas's Hospital Medical SchoolLondonUK

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