Anaesthetic management of infants with glycogen storage disease type II: a physiological approach
- 20 May 2004
- journal article
- Published by Wiley in Pediatric Anesthesia
- Vol. 14 (6) , 514-519
- https://doi.org/10.1111/j.1460-9592.2004.01242.x
Abstract
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.Keywords
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