TY - CHAP M1 - Book, Section TI - Isovaleric Acidemia A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Genetic disorder affecting the branched-chain organic acids, the most frequent of the leucine metabolism disorders. It is a classical type of organic academia. In 50% of cases, the onset of the disease becomes apparent within a few days after birth. This inborn error of metabolism leads to body accumulation of isovaleric acid (and its metabolites) resulting in vomiting, dehydration, severe metabolic acidosis, and neurologic manifestations. A characteristic feature of isovaleric acidemia is a very distinctive and strong odor of sweaty feet. This odor results from accumulation of the compound isovaleric acid. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164074058 ER -