Anesth Pain Med.  2006 Oct;1(2):124-128.

General Anesthesia in a Patient with Infantile Pompe's Disease : A case report

  • 1Department 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.


anesthesia; cardiomyopathy; GSD II; infantile; Pompe's disease
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