Patients with Glycogen storage disease type I (GSD-I) require specialised perioperative management when undergoing any scheduled surgical procedure. The metabolic characteristics of this condition make standard surgical preparation unsafe without a dedicated protocol.
Careful management of glucose and electrolyte levels around the time of surgical procedures is essential in GSD-I. Patients with GSD-I cannot tolerate the typical periods of fasting required before procedures — the routine pre-operative fast that is safe in other patients poses a significant metabolic risk in this population.
The protocol calls for hospital admission on the day before surgery so that continuous intravenous glucose support can be established and maintained throughout the perioperative period. Management continues through the intraoperative phase and into recovery until oral feeding is re-established. The specific intravenous fluid selection, target-guided rate adjustments, and weaning approach are set out in the full protocol.
Target: blood glucose > 70 mg/dlDOI: 10.1038/gim.2014.128
Careful management of the patient's glucose and electrolyte levels around the time of surgical procedures is needed.
Patients with GSD I cannot tolerate typical periods of fasting before procedures.
The patient should be admitted to the hospital on the day before the procedure so that a continuous i.v. supply of glucose can be provided.
The i.v. fluid rate should be adjusted to keep the BG higher than 70 mg/dl.
The i.v. fluids should continue until oral feeding is re-established.
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