05 May 2019 : Case report
Total Intravenous Anesthesia in GLUT1 Deficiency Syndrome Patient: A Case Report
Management of emergency care, Congenital defects / diseases, Educational Purpose (only if useful for a systematic review or synthesis)
Jan Kloka1CDEF, Stefanie Kranepuhl1BCDE, Kai Zacharowski1ACDE, Florian Jürgen Raimann1ABCDEF*DOI: 10.12659/AJCR.914865
Am J Case Rep 2019; 20:647-650
Abstract
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.
Keywords: Anesthesia, Intravenous, Glucose Transporter Type 1, ketogenic diet, Metabolism, Inborn Errors, Monosaccharide Transport Proteins, Rare Diseases, Anesthetics, Intravenous, Carbohydrate Metabolism, Inborn Errors, Child, Fentanyl, mastoidectomy, Neuromuscular Nondepolarizing Agents, Propofol, rocuronium
In Press
12 Feb 2024 : Case report
Diagnostic Challenges and Imaging Considerations for Intraparotid Facial Nerve Schwannoma: A Case Report an...Am J Case Rep In Press; DOI: 10.12659/AJCR.942870
12 Feb 2024 : Case report
Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PETAm J Case Rep In Press; DOI: 10.12659/AJCR.941169
12 Feb 2024 : Case report
Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.942839
13 Feb 2024 : Case report
Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel HematomaAm J Case Rep In Press; DOI: 10.12659/AJCR.943519
Most Viewed Current Articles
10 Jan 2022 : Case report
A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...DOI :10.12659/AJCR.935263
Am J Case Rep 2022; 23:e935263
19 Jul 2022 : Case report
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
05 Mar 2022 : Case report
Acute Limb Ischemia After Self-Injection of Crushed Morphine Tablets into the Radial Artery: Role of Infrar...DOI :10.12659/AJCR.935336
Am J Case Rep 2022; 23:e935336
02 Apr 2022 : Case report
Infective Endocarditis Caused by Pseudomonas luteola in a Pediatric Patient. A Case Report and Review of Li...DOI :10.12659/AJCR.935743
Am J Case Rep 2022; 23:e935743