RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58081167
T1 Isovaleric Acidemia
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58081167
RD 2024/04/19
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.