General Anesthesia in a Patient with Infantile Pompe's Disease : A case report
Anesthesia and Pain Medicine
; : 124-128, 2006.
Article
en Ko
| WPRIM
| ID: wpr-81787
Biblioteca responsable:
WPRO
ABSTRACT
Pompe's disease (type II glycogen storage disease) is an autosomalrecessive disorder caused by a deficiency of lysosomal acid alpha- glucosidase (GAA) leading to the accumulation of glycogen in the lysosomes primarily in cardiac and skeletal muscle. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anesthesia for these patients. The anesthetic management of infant with Pompe's disease presents a variety of challenges. Therefore, understanding the unique cardiac and respiratory physiology is essential to providing safe general anesthesia. We report a case of patient with infantile-onset Pompe's disease who underwent a tracheostomy for a ventilator care.
Palabras clave
Texto completo:
1
Base de datos:
WPRIM
Asunto principal:
Fenómenos Fisiológicos Respiratorios
/
Traqueostomía
/
Enfermedad del Almacenamiento de Glucógeno Tipo II
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Ventiladores Mecánicos
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Músculo Esquelético
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Terapia de Reemplazo Enzimático
/
Glucosidasas
/
Glucógeno
/
Anestesia
/
Anestesia General
Límite:
Humans
/
Infant
Idioma:
Ko
Revista:
Anesthesia and Pain Medicine
Año:
2006
Tipo del documento:
Article