Abstract
The urinary excretion of vanilmandelic acid (VMA), homovanillic acid (HVA), and parahydroxyphenylacetic acid (pHPAA) was measured in 55 children with meningomyelocoele selected at random. In 96% of the children the levels of one or other of these compounds was significantly raised above the normal, usually by a factor of about 3. High VMA levels usually meant high HVA levels but the values for pHPAA appeared to be quite independent of the others. These results suggest a disorder of tyrosine metabolism, and the possible implications are discussed.
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Selected References
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- BOSCOTT R. J., BICKEL H. Detection of some new abnormal metabolites in the urine of phenylketonuria. Scand J Clin Lab Invest. 1953;5(4):380–382. doi: 10.3109/00365515309094216. [DOI] [PubMed] [Google Scholar]
- BOSCOTT R. J., COOKE W. T. Ascorbic acid requirements and urinary excretion of p-hydroxyphenylacetic acid in steatorrhoea and macrocytic anaemia; with a description of the paper-chromatographic technique for identification of hydroxyphenylic acids. Q J Med. 1954 Jul;23(91):307–322. [PubMed] [Google Scholar]
- GJESSING L. R. STUDIES OF FUNCTIONAL NEURAL TUMORS. I. URINARY 3-METHOXY-4-HYDROXY-PHENYL-METABOLITES. Scand J Clin Lab Invest. 1963;15:463–473. [PubMed] [Google Scholar]
- HERMANN G. A. THE DETERMINATION OF URINARY 3-METHOXY-4-HYDORXYMANDELIC (VANILMANDELIC) ACID BY MEANS OF ELECTROPHORESIS WITH CELLULOSE ACETATE MEMBRANE. Am J Clin Pathol. 1964 Apr;41:373–376. doi: 10.1093/ajcp/41.4.373. [DOI] [PubMed] [Google Scholar]
- LEVIN E. Y., LEVENBERG B., KAUFMAN S. The enzymatic conversion of 3,4-dihydroxyphenylethylamine to norepinephrine. J Biol Chem. 1960 Jul;235:2080–2086. [PubMed] [Google Scholar]
- McKibbin B., Porter R. W. The incidence of vitamin-C deficiency in meningomyelocele. Dev Med Child Neurol. 1967 Jun;9(3):338–344. doi: 10.1111/j.1469-8749.1967.tb02278.x. [DOI] [PubMed] [Google Scholar]
- O'Gorman L. P. The estimation of vanilmandelic acid (VMA) in serum and urine by a radioactive 14C labelling technique. Clin Chim Acta. 1968 Mar;19(3):485–492. doi: 10.1016/0009-8981(68)90276-3. [DOI] [PubMed] [Google Scholar]
- TOMPSETT S. L. A study of the indentification and determination of phenols and phenolic acids in urine. Clin Chim Acta. 1958 Mar;3(2):149–159. doi: 10.1016/0009-8981(58)90073-1. [DOI] [PubMed] [Google Scholar]