Perioperative anesthetic management of a patient with glycogen storage disease type III Glikojen depo hastaliǧi tip III'lü olguda perioperatif anestezik yaklaşim


BİÇER C. , Öner M. , ÜLGEY A. , Boyaci A.

Erciyes Tip Dergisi, vol.30, no.4, pp.288-291, 2008 (Journal Indexed in SCI Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 30 Issue: 4
  • Publication Date: 2008
  • Title of Journal : Erciyes Tip Dergisi
  • Page Numbers: pp.288-291

Abstract

Glycogen storage disease type III results from a deficiency of the enzyme, amylo-1,6-glucosidase, which is responsible for the breakdown of debranching of the glycogen molecule during catabolism and goes with hypoglycaemia, muscle weakness and endorgan dysfunction due to glycogen accumulation. Primary problems for anaesthesiologist are hypoglycaemia, liver dysfunction, delayed anaesthetic recovery, excessive surgical bleeding and cardiomyopathy. We present the anaesthetic management of a patient with glycogen storage disease type III scheduled for orthopedic surgery.