This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on Web of Science (9)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

HANS MEYER, M.D.; EDWIN T. MERTZ, Ph.D.; HAROLD E. STADLER, M.D.; HENRY LELAND, Ph.D.; JOSEPH CALANDRO, M.T.

AMA Arch Intern Med. 1958;101(6):1094-1105.

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

The extreme interest which has been shown in the use of special diet in the attempt to control or prevent nervous system degeneration associated with phenylketonuria may be due to the obvious fact that at present no other practical approach to the problem exists. The rather widespread discussion of the difficulties associated with treatment of the phenylketonuric has led to more extensive testing for the disorder and concomitant more accurate and early diagnosis.

Transmitted as a recessive hereditary trait apparently linked with blondism,¹ phenylketonuria results from the inborn failure of the organism to hydroxylate phenylalanine to tyrosine. The accumulated phenylalanine is partially deaminated in the kidney and excreted in the urine as phenylpyruvic acid, phenyllactic acid, and phenylacetic acid. The plasma concentration of phenylalanine in the normal human is approximately 1.4 mg. per 100 cc. Plasma levels of phenylalanine in the phenylpyruvic person are found to be 20 to . . . [Full Text PDF of this Article]

Author Affiliations
Butlerville, Ind.

Footnotes
Submitted for publication Aug. 23, 1957.

Aided by a grant from the U. S. Public Health Service, National Institute of Neurological Disease and Blindness (677D).

The personnel of Muscatatuck State School Butlerville, Ind., aided in the completion of this study (Dr. Alfred Sasser, Jr., Administrator).

Muscatatuck State School, Consultant in Biochemistry (Dr. Mertz), Consultant in Pediatric Neurology (Dr. Stadler), and Chief of Laboratory Services (Mr. Calandro). Fort Wayne State School, Fort Wayne, Ind., Clinical Director (Dr. Meyer). Parsons State Hospital and Training Center, Parsons, Kan., Director of Psychological Services (Dr. Leland).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?