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. 1963 Oct;38(201):485–491. doi: 10.1136/adc.38.201.485

Dietary Treatment of a Child with Maple Syrup Urine Disease (Branched-chain Ketoaciduria)

R G Westall
PMCID: PMC2019098  PMID: 14065992

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bloxam H. R., Day M. G., Gibbs N. K., Woolf L. I. An inborn defect in the metabolism of tyrosine in infants on a normal diet. Biochem J. 1960 Nov;77(2):320–326. doi: 10.1042/bj0770320. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. DANCIS J., LEVITZ M., MILLER S., WESTALL R. G. Maple syrup urine disease. Br Med J. 1959 Jan 10;1(5114):91–93. doi: 10.1136/bmj.1.5114.91. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. DENT C. E., WESTALL R. G. Studies in maple syrup urine disease. Arch Dis Child. 1961 Jun;36:259–268. doi: 10.1136/adc.36.187.259. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. GULLINO P., WINITZ M., BIRNBAUM S. M., CORNFIELD J., OTEY M. C., GREENSTEIN J. P. Studies on the metabolism of amino acids and related compounds in vivo. I. Toxicity of essential amino acids, individually and in mixtures, and the protective effect of L-arginine. Arch Biochem Biophys. 1956 Oct;64(2):319–332. doi: 10.1016/0003-9861(56)90276-4. [DOI] [PubMed] [Google Scholar]
  5. Levine S. Z., Gordon H. H., Marples E. A DEFECT IN THE METABOLISM OF TYROSINE AND PHENYLALANINE IN PREMATURE INFANTS. II. SPONTANEOUS OCCURRENCE AND ERADICATION BY VITAMIN C. J Clin Invest. 1941 Mar;20(2):209–219. doi: 10.1172/JCI101213. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. MACKENZIE D. Y., WOOLF L. I. Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency. Br Med J. 1959 Jan 10;1(5114):90–91. doi: 10.1136/bmj.1.5114.90. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. MENKES J. H., HURST P. L., CRAIG J. M. A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance. Pediatrics. 1954 Nov;14(5):462–467. [PubMed] [Google Scholar]
  8. MENKES J. H. Maple syrup disease; isolation and identification of organic acids in the urine. Pediatrics. 1959 Feb;23(2):348–353. [PubMed] [Google Scholar]
  9. MOORE S., STEIN W. H. Procedures for the chromatographic determination of amino acids on four per cent cross-linked sulfonated polystyrene resins. J Biol Chem. 1954 Dec;211(2):893–906. [PubMed] [Google Scholar]
  10. NORTON P. M., ROITMAN E., SNYDERMAN S. E., HOLT L. E., Jr A new finding in maple-syrup-urine disease. Lancet. 1962 Jan 6;1(7219):26–27. doi: 10.1016/s0140-6736(62)92646-6. [DOI] [PubMed] [Google Scholar]
  11. PIEZ K. A., MORRIS L. A modified procedure for the automatic analysis of amino acids. Anal Biochem. 1960 Nov;1:187–201. doi: 10.1016/0003-2697(60)90045-2. [DOI] [PubMed] [Google Scholar]

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