Glycogen Storage Disorders

 

 

Background 

 

  • Inherited disorders caused by abnormalities of enzymes that regulate glycogen synthesis & breakdown

  • 3 common features with all these disorders: 

    • Acidosis secondary to fat & protein metabolism

    • Risk of hypoglycemia secondary to the failure to metabolize glycogen to glucose

    • Cardiac & hepatic dysfunction secondary to destruction of normal tissue by accumulated glycogen

 

 

Considerations

 

  • Careful monitoring of glucose & give D5W during fasting periods 

  • Careful evaluation of cardiac & hepatic function 

  • Careful review of previous anesthetics & obtain endocrinology consultation before surgery 

  • Monitor glucose & pH intra-op

  • Avoid lactate containing solutions (they cannot convert lactate to glycogen)