RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164069515 T1 Familial Hyperlysinemia T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164069515 RD 2023/03/30 AB The enzyme deficiencies of α-aminoadipic semialdehyde dehydrogenase and the saccharopine dehydrogenases have been associated with increased serum levels of L-lysine. The clinical presentation is very variable and may include developmental delay, hypotonia, lethargy recurrent emesis, and diarrhea. The use of anesthetics deemed to be safe in malignant hyperthermia (MH)-susceptible patients produced an uneventful perioperative course.