Bibliographic Details
| 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 |
| Source: |
JA Clinical Reports, Vol 8, Iss 1, Pp 1-4 (2022) |
| Publisher Information: |
SpringerOpen, 2022. |
| Publication Year: |
2022 |
| Collection: |
LCC:Anesthesiology
LCC:Medical emergencies. Critical care. Intensive care. First aid |
| Subject Terms: |
Pediatric, Urea cycle disorders, UCD, Carbamoyl phosphate synthetase deficiency, CPS1D, Hyperammonemia, Anesthesiology, RD78.3-87.3, Medical emergencies. Critical care. Intensive care. First aid, RC86-88.9 |
| More Details: |
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. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
2363-9024 |
| Relation: |
https://doaj.org/toc/2363-9024 |
| DOI: |
10.1186/s40981-022-00558-9 |
| Access URL: |
https://doaj.org/article/c914a2ed4a71471f91f95a403e2c81f7 |
| Accession Number: |
edsdoj.914a2ed4a71471f91f95a403e2c81f7 |
| Database: |
Directory of Open Access Journals |
