TY - CHAP M1 - Book, Section TI - Isovaleric Acidemia A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Marciniak, Bruno A1 - Dalens, Bernard J. PY - 2006 T2 - Syndromes: Rapid Recognition and Perioperative Implications AB - Genetic disorder affecting the branched-chain organic acids, the most frequent of the leucine metabolism disorders. 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. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/19 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=58081167 ER -