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

Richard J Ing1, D Ryan Cook, Resai A Bengur

  • 1Department of Anesthesiology, Duke University Medical Center, Durham, NC 27710, USA. ing00001@mc.duke.edu

Paediatric Anaesthesia
|May 22, 2004
PubMed

Insights

Pompe disease (Glycogen Storage Disease type II) affects heart and muscle. New enzyme therapy improves outcomes, making anesthesia safer. Understanding cardiac changes in GSD-II is crucial for patient care.

Area of Science:

  • Genetics
  • Cardiology
  • Anesthesiology

Background:

  • Pompe disease (Glycogen Storage Disease type II) is a genetic disorder impacting cardiac and skeletal muscles.
  • Infantile GSD-II historically led to early mortality from cardiac and respiratory failure.
  • Recent advancements in enzyme replacement therapy (ERT) have improved patient outcomes.

Purpose of the Study:

  • To highlight the importance of understanding cardiac physiology in GSD-II patients.
  • To emphasize the need for safe general anesthesia in the context of improved clinical outcomes.
  • To provide essential knowledge for clinicians managing GSD-II patients undergoing anesthesia.

Main Methods:

  • Review of existing literature on Pompe disease and cardiac manifestations.
  • Analysis of physiological changes in GSD-II patients.
  • Synthesis of information relevant to anesthetic management.

Main Results:

  • Enzyme replacement therapy has significantly improved survival and clinical status.
  • Patients with GSD-II exhibit unique cardiac alterations requiring careful consideration.
  • Anesthetic management requires a thorough understanding of these cardiac changes.

Conclusions:

  • Improved outcomes in GSD-II necessitate updated anesthetic protocols.
  • Knowledge of GSD-II cardiac physiology is vital for safe anesthetic practice.
  • Multidisciplinary care is essential for optimizing patient safety and outcomes.

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