Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.

Journal Article (Journal Article)

Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anaesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anaesthesia.

Full Text

Duke Authors

Cited Authors

  • Ing, RJ; Cook, DR; Bengur, RA; Williams, EA; Eck, J; Dear, GDL; Ross, AK; Kern, FH; Kishnani, PS

Published Date

  • June 2004

Published In

Volume / Issue

  • 14 / 6

Start / End Page

  • 514 - 519

PubMed ID

  • 15153218

International Standard Serial Number (ISSN)

  • 1155-5645

Digital Object Identifier (DOI)

  • 10.1111/j.1460-9592.2004.01242.x


  • eng

Conference Location

  • France