Isovalerylglycine

Isovalerylglycine is a compound detected in urine that indicates isovaleric acidemia, a rare genetic metabolic disorder. This condition impairs the breakdown of the amino acid leucine, causing toxic organic acids to accumulate in the body. Elevated isovalerylglycine levels cause neurological problems, developmental delays, and a characteristic 'sweaty feet' odor. This test is typically performed on newborn screening or in infants with developmental concerns. Early detection allows dietary management to prevent serious complications.