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JA Clinical Reports
Published in: JA Clinical Reports 1/2022

Open Access 01-12-2022 | Liver Transplantation | Case report

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

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

Published in: JA Clinical Reports | Issue 1/2022

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Abstract

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.
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Metadata
Title
Anesthesia management in living-donor liver transplantation in a patient with carbamoyl phosphate synthetase deficiency: a case report
Authors
Hiroki Matsushita
Tetsushiro Fujiyoshi
Koichiro Yoshimaru
Toshiharu Matsuura
Yuichi Mushimoto
Yuji Karashima
Ken Yamaura
Publication date
01-12-2022
Publisher
Springer Berlin Heidelberg
Published in
JA Clinical Reports / Issue 1/2022
Electronic ISSN: 2363-9024
DOI
https://doi.org/10.1186/s40981-022-00558-9

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