Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.
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.
Ing, RJ; Cook, DR; Bengur, RA; Williams, EA; Eck, J; Dear, GDL; Ross, AK; Kern, FH; Kishnani, PS
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