RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58082146
T1 Ornithine Carbamoyltransferase Deficiency (OTCD)
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=58082146
RD 2023/03/25
AB Rare  genetic  disorder  characterized  by  complete  or  partial  lack  of  the  enzyme  ornithine  transcarbamylase  (OTC).  Lack  of  the  enzyme  results  in  excessive  hyperammonemia,  which  is  known  as  a  neurotoxin.  Clinically,  patients  present  vomiting,  refusal  to  eat,  progressive  lethargy,  and  coma.