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
Richard J
RJ
Department of Anesthesiology, Duke University Medical Center, Durham, NC 27710, USA. ing00001@mc.duke.edu
Cook
D Ryan
DR
Bengur
Resai A
RA
Williams
Eric A
EA
Eck
John
J
Dear
Guy de L
Gde L
Ross
Allison K
AK
Kern
Frank H
FH
Kishnani
Priya S
PS
D000768:Anesthesia, General / Q000379:methods*; D006009:Glycogen Storage Disease Type II / Q000188:drug therapy / Q000503:physiopathology; D006321:Heart / Q000503:physiopathology; D006801:Humans; D007223:Infant; D011994:Recombinant Proteins / Q000627:therapeutic use; D000520:alpha-Glucosidases / Q000627:therapeutic use
D011994:Recombinant Proteins; D000520:alpha-Glucosidases