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Perioperative Management of a Child With Glucose Transporter Type 1 Deficiency Syndrome: A Case Report

Tsubasa Yoshida1, Kazuyoshi Shimizu2, Satoshi Suzuki2

  • 1From the Department of Anesthesiology, Tottori City Hospital, Tottori, Japan.

A&A Practice
|April 11, 2018
PubMed

Insights

Glucose transporter type 1 deficiency syndrome (GLUT1DS) impairs brain glucose transport, causing epilepsy. Perioperative management focuses on ketone bodies, metabolic acidosis, and surgical stress to prevent seizures in GLUT1DS patients.

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Pediatric Care

Background:

  • Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare genetic disorder.
  • It impairs glucose transport to the brain, leading to neurological dysfunction.
  • Intractable epilepsy is a common and severe symptom of GLUT1DS.

Observation:

  • The study reports on the perioperative management of a pediatric patient diagnosed with GLUT1DS.
  • The focus was on addressing the specific metabolic and neurological challenges posed by the condition during a surgical period.

Findings:

  • Successful perioperative management strategies were implemented for a child with GLUT1DS.
  • Key interventions included maintaining adequate ketone body concentrations.
  • Compensation of metabolic acidosis and reduction of surgical stress were also crucial.

Implications:

  • This case highlights the importance of a multidisciplinary approach to managing GLUT1DS patients undergoing surgery.
  • Effective perioperative care can help prevent seizures and ensure brain energy supply in GLUT1DS.
  • The findings contribute to the understanding of managing rare metabolic encephalopathies.

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