Anesthesia management in living-donor liver transplantation in a patient with carbamoyl phosphate synthetase deficiency: a case report

Hiroki Matsushita, Tetsushiro Fujiyoshi, Koichiro Yoshimaru, Toshiharu Matsuura, Yuichi Mushimoto, Yuji Karashima, Ken Yamaura

研究成果: ジャーナルへの寄稿学術誌査読

抄録

Background: Carbamoyl phosphate synthetase deficiency (CPS1D) is a urea-cycle disorder (UCD). We report successful perioperative management of pediatric living donor liver transplantation (LDLT) in a CPS1D patient. Case presentation: A 10-year-old female patient with CPS1D underwent LDLT. Proper administration of dextrose 50% and 60 kcal/kg/day with l-arginine and l-carnitine resulted in the avoidance of intraoperative hyperammonemia induced by hypercatabolism. Serum ammonia level transiently increased to 61 mmol/L in the anhepatic phase and decreased to 44 mmol/L after reperfusion. Conclusions: We suggest anesthesia management with administration of dextrose to avoid hyperammonemia during LDLT in patients with CPS1D.

本文言語英語
論文番号71
ジャーナルJA Clinical Reports
8
1
DOI
出版ステータス出版済み - 12月 2022

!!!All Science Journal Classification (ASJC) codes

  • 麻酔学および疼痛医療

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