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Anaesthetic management of infants with glycogen storage disease type II: a physiological approach.
Ing, Richard J; Cook, D Ryan; Bengur, Resai A; Williams, Eric A; Eck, John; Dear, Guy de L; Ross, Allison K; Kern, Frank H; Kishnani, Priya S.
Afiliación
  • Ing RJ; Department of Anesthesiology, Duke University Medical Center, Durham, NC 27710, USA. ing00001@mc.duke.edu
Paediatr Anaesth ; 14(6): 514-9, 2004 Jun.
Article en En | MEDLINE | ID: mdl-15153218
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.
Asunto(s)
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Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno Tipo II / Anestesia General Límite: Humans / Infant Idioma: En Revista: Paediatr Anaesth Asunto de la revista: ANESTESIOLOGIA / PEDIATRIA Año: 2004 Tipo del documento: Article País de afiliación: Estados Unidos
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Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno Tipo II / Anestesia General Límite: Humans / Infant Idioma: En Revista: Paediatr Anaesth Asunto de la revista: ANESTESIOLOGIA / PEDIATRIA Año: 2004 Tipo del documento: Article País de afiliación: Estados Unidos