A patient with glycogen storage disease Type 1b (a variant of Type 1a, or von Gierke Disease) is at risk for a number of problems in the perioperative period. Recognition of potential problems allows for adequate monitoring and early interventions.
Metabolic defect: Deficiency of glucose 6-phosphate microsomal translocase, which is the transport protein for glucose 6-phosphate released from glycogen
Inheritance: autosomal recessive
Parameter
Problem
Solution
mental retardation
behavioral problems during induction and recovery
additional nursing assistance, restraints as appropriate, sedation
hypoglycemia
perioperative convulsions
reduce preoperative fasting, monitor levels during surgery, IV infusion glucose solutions
lactic acidosis
metabolic acidosis
monitor arterial pH, avoid lactated Ringer's solution, mechanical ventilation to prevent respiratory acidosis, monitor with capnography, administer sodium bicarbonate IV
hyperventilation
respiratory alkalosis can result in release of lactate from skeletal muscle
monitor arterial pH, mechanical ventilation
coagulopathy associated with platelet dysfunction
excessive bleeding
control of glucose levels, intraoperative hemostasis
hepatomegaly
elevation of the diaphragm reduces lung expansion
may require mechanical ventilation
neutropenia and neutrophil dysfunction
post-operative infection
aseptic technique
Glucose is infused intravenously at a rate of 4-8 mg per kg per minute.
To read more or access our algorithms and calculators, please log in or register.
