Two different anesthetic managements of a patient with von Gierke's disease.

Abstract

Von Gierke's disease, a form of glycogen storage disturbance, is a rare metabolic disorder with important implications for anesthesiologists. It is caused by the lack of the glucose-6-phosphatase, which is necessary for the liver to convert glycogen to glucose. To avoid severe hypoglycemia, it is crucial to keep oral feeding at intervals 2-3 hr for maintaining a normal blood sugar level. The metabolic derangements of von Gierke's disease may result in serious complications in patients undergoing surgery and anesthesia. We report the anesthetic managements of a patient with von Gierke's disease in two operations with different encounters.

• Authors: I-Ren Huang, Wei-Horng Jean, Cheng-Wei Lu, Chia-Chan Wu, Tzu-Yu Lin, Yueh-Hsun Chuang, Wei-Zen Sun
• Journal: Acta anaesthesiologica Taiwanica : official journal of the Taiwan Society of Anesthesiologists (Acta Anaesthesiol Taiwan) Vol. 44 Issue 1 Pg. 51-3 (Mar 2006) ISSN: 1875-4597 [Print] China (Republic : 1949- )
• PMID: 16623410 (Publication Type: Case Reports, Journal Article)

Chemical References

• Blood Glucose

Topics

• Adult
• Anesthesia (methods)
• Blood Glucose (analysis)
• Glycogen Storage Disease Type I (metabolism)
• Humans
• Male