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





mental retardation

behavioral problems during induction and recovery

additional nursing assistance, restraints as appropriate, sedation


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


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


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.