A Simple Screening Method for Detecting Isovalerylglycine in Urine of Patients with Isovaleric Acidemia
Keyword(s):
Abstract Isovaleric acidemia is one of an enlarging group of inherited metabolic diseases now recognized to cause death in the neonatal period. Rapid, simple detection is essential for diagnosis of these conditions. We describe a method that fulfills these criteria for isovaleric acidemia. Excretion of isovalerylglycine in the urine more reliably indicates the condition than the presence of volatile isovaleric acid in the blood. Isovalerylglycine is extracted from urine and separated by thin-layer chromatography from other constituents of urine. It is detected by spraying the chromatogram with bromcresol purple. Isovalerylglycine does not appear in normal urine.
1978 ◽
Vol 89
(2)
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pp. 231-238
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1977 ◽
Vol 60
(6)
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pp. 1369-1371
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1998 ◽
Vol 81
(4)
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pp. 844-847
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1969 ◽
Vol 42
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pp. 83-88
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1975 ◽
Vol 58
(1)
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pp. 44-47
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