General Anesthesia in a Patient with Infantile Pompe's Disease : A case report
Anesthesia and Pain Medicine
; : 124-128, 2006.
Article
in Ko
| WPRIM
| ID: wpr-81787
Responsible library:
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Respiratory Physiological Phenomena
/
Tracheostomy
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Glycogen Storage Disease Type II
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Ventilators, Mechanical
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Muscle, Skeletal
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Enzyme Replacement Therapy
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Glucosidases
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Glycogen
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Anesthesia
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Anesthesia, General
Limits:
Humans
/
Infant
Language:
Ko
Journal:
Anesthesia and Pain Medicine
Year:
2006
Type:
Article