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

Publication ,  Journal Article
Ing, RJ; Cook, DR; Bengur, RA; Williams, EA; Eck, J; Dear, GDL; Ross, AK; Kern, FH; Kishnani, PS
Published in: Paediatr Anaesth
June 2004

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.

Duke Scholars

Published In

Paediatr Anaesth

DOI

ISSN

1155-5645

Publication Date

June 2004

Volume

14

Issue

6

Start / End Page

514 / 519

Location

France

Related Subject Headings

  • alpha-Glucosidases
  • Recombinant Proteins
  • Infant
  • Humans
  • Heart
  • Glycogen Storage Disease Type II
  • Anesthesiology
  • Anesthesia, General
  • 3213 Paediatrics
  • 3202 Clinical sciences
 

Citation

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Chicago
ICMJE
MLA
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Ing, R. J., Cook, D. R., Bengur, R. A., Williams, E. A., Eck, J., Dear, G. D. L., … Kishnani, P. S. (2004). Anaesthetic management of infants with glycogen storage disease type II: a physiological approach. Paediatr Anaesth, 14(6), 514–519. https://doi.org/10.1111/j.1460-9592.2004.01242.x
Ing, Richard J., D Ryan Cook, Resai A. Bengur, Eric A. Williams, John Eck, Guy de L. Dear, Allison K. Ross, Frank H. Kern, and Priya S. Kishnani. “Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.Paediatr Anaesth 14, no. 6 (June 2004): 514–19. https://doi.org/10.1111/j.1460-9592.2004.01242.x.
Ing RJ, Cook DR, Bengur RA, Williams EA, Eck J, Dear GDL, et al. Anaesthetic management of infants with glycogen storage disease type II: a physiological approach. Paediatr Anaesth. 2004 Jun;14(6):514–9.
Ing, Richard J., et al. “Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.Paediatr Anaesth, vol. 14, no. 6, June 2004, pp. 514–19. Pubmed, doi:10.1111/j.1460-9592.2004.01242.x.
Ing RJ, Cook DR, Bengur RA, Williams EA, Eck J, Dear GDL, Ross AK, Kern FH, Kishnani PS. Anaesthetic management of infants with glycogen storage disease type II: a physiological approach. Paediatr Anaesth. 2004 Jun;14(6):514–519.
Journal cover image

Published In

Paediatr Anaesth

DOI

ISSN

1155-5645

Publication Date

June 2004

Volume

14

Issue

6

Start / End Page

514 / 519

Location

France

Related Subject Headings

  • alpha-Glucosidases
  • Recombinant Proteins
  • Infant
  • Humans
  • Heart
  • Glycogen Storage Disease Type II
  • Anesthesiology
  • Anesthesia, General
  • 3213 Paediatrics
  • 3202 Clinical sciences