Total intravenous anesthesia in GLUT1 deficiency syndrome patient : a case report
- 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.
Author: | Jan Andreas KlokaORCiDGND, Stefanie Kranepuhl, Kai ZacharowskiORCiDGND, Florian RaimannORCiDGND |
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URN: | urn:nbn:de:hebis:30:3-510123 |
DOI: | https://doi.org/10.12659/AJCR.914865 |
ISSN: | 1941-5923 |
Pubmed Id: | https://pubmed.ncbi.nlm.nih.gov/31055589 |
Parent Title (English): | The American journal of case reports |
Publisher: | International Scientific Literature |
Place of publication: | New York, NY |
Document Type: | Article |
Language: | English |
Year of Completion: | 2019 |
Date of first Publication: | 2019/05/05 |
Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
Release Date: | 2019/09/12 |
Tag: | Anesthesia, Intravenous; Glucose Transporter Type 1; Ketogenic Diet; Metabolism, Inborn Errors; Monosaccharide Transport Proteins; Rare Diseases |
Volume: | 20 |
Page Number: | 4 |
First Page: | 647 |
Last Page: | 650 |
Note: | 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. |
HeBIS-PPN: | 454015186 |
Institutes: | Medizin / Medizin |
Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
Sammlungen: | Universitätspublikationen |
Open-Access-Publikationsfonds: | Medizin |
Licence (German): | Creative Commons - Namensnennung-Nicht kommerziell - Keine Bearbeitung 4.0 |