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Paediatric anaesthesia · Apr 2024
Case ReportsChanges in branched-chain amino acids in an infant with maple syrup urine disease during perioperative pediatric liver transplant: A case report.
- Pipat Saeyup, Chomchanat Tubjaroen, Wuttichart Kamolvisit, Voranush Chongsrisawat, and Winnie Thaveepunsan.
- Department of Anesthesiology, King Chulalongkorn Memorial Hospital, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
- Paediatr Anaesth. 2024 Apr 1; 34 (4): 366370366-370.
AbstractAn 11-month-old female infant diagnosed with classic subtype IB maple syrup urine disease underwent living donor liver transplantation. Blood samples for plasma amino acid analysis were collected during the three phases of the operation. Despite the perioperative prophylactic administration of 12.5% hypertonic dextrose solution with insulin and a 20% intralipid emulsion, the blood levels of the branched-chain amino acids increased dramatically during surgery, consistent with an acute intraoperative metabolic decompensation. However, these blood levels normalized soon after liver transplantation with an excellent outcome. We suggest that the occurrence of an intraoperative metabolic crisis during liver transplantation is not necessarily a sign of graft failure.© 2024 John Wiley & Sons Ltd.
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