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Obstetric Anesthesia
Anesthesia and Pain Medicine 2006;1(2):120-123.
Published online October 30, 2006.
General Anesthesia in a Patient with Infantile Pompe's Disease : A case report
Young Jae Lee, Woo Suk Chung, Jung Hyun Lee, Soo Chang Son
Department of Anesthesiology and Pain Medicine, College of Medicine, Chungnam National University, Daejeon, Korea.
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: anesthesia, cardiomyopathy, GSD II, infantile, Pompe's disease

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