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Clinical Chemistry, Vol 8, 242-245, Copyright © 1962 by the American Association for Clinical Chemistry
1 Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati 29, Ohio.
Urine specimens dried on filter paper squares were obtained from 700 mentally retarded children. A microbiological assay was used to screen the specimens for the presence of valine, leucine, and isoleucine. Three specimens gave positive tests for all three amino acids. A generalized aminoaciduria was observed in two specimens, and in the third, elevation of valine and leucine/isoleucine was confirmed by paper chromatography. The use of these screening procedures for early detection of children with maple sugar urine disease is suggested.
Submitted on June 5, 1961
The following articles in journals at HighWire Press have cited this article:
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M. H. Fogelson How to Detect Maple-Syrup Urine Disease in Newborn Infants Clinical Pediatrics, September 1, 1970; 9(9): 538 - 538. [PDF] |
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