The Relationship between the Branched Chain Amino Acids and Their α-Ketoacids in Maple Syrup Urine Disease
作者:
SELMA SNYDERMAN,
FRED GOLDSTEIN,
CLAUDE SANSARICQ,
PATRICIA NORTON,
期刊:
Pediatric Research
(OVID Available online 1984)
卷期:
Volume 18,
issue 9
页码: 851-853
ISSN:0031-3998
年代: 1984
出版商: OVID
数据来源: OVID
摘要:
SummaryPlasma branched chain amino acid levels and their respective ketoacid analogues were determined in seven maple syrup urine disease patients ranging in age from 12 h to 12 years. One hundred one pairs were analyzed. There was a high degree of correlation between the amino acid and its ketoacid analogue at every amino acid level. The coefficient of correlation (0.84) was highest for leucine-α-ketoisocaproic acid. The ratio of ketoacid analogue to amino acid, (0.87), was also the greatest for leucine. The close correlation implies that adequate monitoring for therapy can be accomplished by the use of the technically simpler and more rapid determination of the plasma branched chain amino acids.
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