Abstract
Glucose transporter type 1 deficiency syndrome (GLUT1DS) causes central nervous system dysfunction including intractable epilepsy caused by impaired glucose transport to the brain. To prevent convulsions and maintain an energy source for the brain in patients with GLUT1DS, the maintenance of adequate ketone body concentrations, compensation of metabolic acidosis, and reduction of surgical stress are essential. We here report the perioperative management of a child with GLUT1DS.
MeSH terms
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Blood Glucose
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Carbohydrate Metabolism, Inborn Errors / blood
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Carbohydrate Metabolism, Inborn Errors / drug therapy
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Carbohydrate Metabolism, Inborn Errors / surgery*
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Carbohydrate Metabolism, Inborn Errors / urine
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Child, Preschool
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Female
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Humans
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Isotonic Solutions / therapeutic use
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Ketone Bodies / urine
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Monosaccharide Transport Proteins / blood
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Monosaccharide Transport Proteins / deficiency*
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Monosaccharide Transport Proteins / urine
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Perioperative Care
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Perioperative Period
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Sodium Bicarbonate / therapeutic use
Substances
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Blood Glucose
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Isotonic Solutions
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Ketone Bodies
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Monosaccharide Transport Proteins
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Ringer's acetate
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Sodium Bicarbonate
Supplementary concepts
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Glut1 Deficiency Syndrome