TY  - JOUR
A1  - Kloka, Jan Andreas
A1  - Kranepuhl, Stefanie
A1  - Zacharowski, Kai
A1  - Raimann, Florian
T1  - Total intravenous anesthesia in GLUT1 deficiency syndrome patient : a case report
T2  - The American journal of case reports
N2  - Background: GLUT1-deficiency-syndrome (G1DS) is an autosomal dominant genetic disorder based on a mutation of the SLC2A1 gene. This mutation can lead to an encephalopathy due to abnormal glucose transport in the brain. G1DS is a rare disease, with an estimated incidence of 1: 90 000.
Case report: We report a case of a 10-year-old female who presented with recurrent fever, headaches, and vertigo for more than 3 days within 2 weeks following pneumonia. A bilateral mastoiditis was proven by a cerebral magnetic resonance imaging and a cranial computed tomography scan. The patient had to undergo mastoidectomy and thus, her first general anesthesia. Half a year previously she was diagnosed with G1DS. According to the standard of care, a ketogenic diet had been administered since the patient’s diagnosis 6 months earlier. Our patient received a total intravenous anesthesia (TIVA) using propofol, fentanyl, and rocuronium administered without any incidents.
Conclusions: We recommend normoglycemia during the perioperative phase and avoidance of glucose-based medication to keep a patient’s ketotic state. Our case highlights that TIVA, with the outlined medication used in this case, was safe when the patient’s ketotic state and periprocedural blood glucose was monitored continuously. Nevertheless, we would suggest using remifentanil instead of fentanyl for future TIVAs due to a reduced increase in blood glucose level in our patient.
KW  - Anesthesia, Intravenous
KW  - Glucose Transporter Type 1
KW  - Ketogenic Diet
KW  - Metabolism, Inborn Errors
KW  - Monosaccharide Transport Proteins
KW  - Rare Diseases
Y1  - 2019
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/51012
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-510123
SN  - 1941-5923
N1  - This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
VL  - 20
SP  - 647
EP  - 650
PB  - International Scientific Literature
CY  - New York, NY
ER  -