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KMID : 1155520060010020124
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Anesthesia and Pain Medicine
2006 Volume.1 No. 2 p.124 ~ p.128
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General Anesthesia in a Patient with Infantile Pompe’s Disease -A case report-
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Lee Young-Jae
Chung Woo-Suk
Lee Jung-Hyun
Son Soo-Chang
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Abstract
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Pompe’s disease (type II glycogen storage disease) is an autosomal recessive disorder caused by a deficiency of lysosomal acid α- 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.(Anesth Pain Med 2006; 1: 124∼128)
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KEYWORD
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anesthesia, cardiomyopathy, GSD II, infantile, Pompe’s disease.
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Listed journal information
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