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Studies in Infants on High-Protein Diets

Harvey L. Levy, MD; Vivian E. Shih, MD; Phyllis M. Madigan, AB; Valerie Karolkewicz, AB; Jane R. Carr, BS; Ann Lum, BA; Agnes A. Richards, BS; John D. Crawford, MD; Robert A. MacCready, MD

Am J Dis Child. 1969;117(1):96-103.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

HYPERMETHIONINEMIA in infancy occurs regularly with homocystinuria due to cystathionine synthase deficiency.^1,2 It has also been seen in association with hereditary tyrosinemia³ and with neonatal hepatitis.⁴ Interest in the early detection of hypermethioninemia has been stimulated by the fact that homocystinuria due to cystathionine synthase deficiency is one of the more common inborn errors of amino acid metabolism and by the possibility that brain damage in this condition may be prevented by a special low-methionine diet.¹

In 1966 the Massachusetts Metabolic Screening Program conducted by the State Laboratory Institute, Massachusetts Department of Public Health, incorporated blood filter paper chromatography for the detection of hypermethioninemia and other hyperaminoacidemias in newborn and 4- to 10-week-old infants.^5,6 In January 1968, the Guthrie bacterial inhibition assay for methionine (R. Guthrie, unpublished data) was included as a standard test for all blood specimens received in the Massachusetts screening program. These . . . [Full Text PDF of this Article]

Author Affiliations

From the State Laboratory Institute, Massachusetts Department of Public Health (Phyllis M. Madigan; Valerie Karolkewicz; Jane R. Carr; Ann Lum; and Dr. MacCready); the departments of neurology and pediatrics, Harvard Medical School; and the Joseph P. Kennedy, Jr., Memorial Laboratories at the Massachusetts General Hospital (Drs. Levy, Shih, and Crawford; and Agnes Richards), Boston.

Footnotes

Presented in part before the American Pediatric Society, Atlantic City, NJ, May 1-4, 1968.

Submitted by the authors for the Mary Efron Memorial Issue of the JOURNAL.

Reprint requests to Massachusetts General Hospital, Neurology Research, Boston 02114 (Dr. Levy).

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