Matsushita Hiroki, Fujiyoshi Tetsushiro, Yoshimaru Koichiro, Matsuura Toshiharu, Mushimoto Yuichi, Karashima Yuji, Yamaura Ken
Department of Anesthesiology and Critical Care Medicine, Kyushu University Hospital, 3-1-1 Maidashi Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Pediatric Surgery, Kyushu University Hospital, 3-1-1 Maidashi Higashi-ku, Fukuoka, 812-8582, Japan.
JA Clin Rep. 2022 Sep 7;8(1):71. doi: 10.1186/s40981-022-00558-9.
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.
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.
We suggest anesthesia management with administration of dextrose to avoid hyperammonemia during LDLT in patients with CPS1D.
氨甲酰磷酸合成酶缺乏症（CPS1D）是一种尿素循环障碍（UCD）。我们报告了1例CPS1D患者小儿活体肝移植（LDLT）围手术期的成功管理。
一名10岁CPS1D女性患者接受了LDLT。适当给予50%葡萄糖和60千卡/千克/天的L-精氨酸和L-肉碱可避免因高分解代谢引起的术中高氨血症。无肝期血清氨水平短暂升至61毫摩尔/升，再灌注后降至44毫摩尔/升。
我们建议在CPS1D患者LDLT期间给予葡萄糖进行麻醉管理以避免高氨血症。
