Anesth Pain Med Search

CLOSE


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. scson@cnu.ac.kr
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: anesthesia, cardiomyopathy, GSD II, infantile, Pompe's disease


ABOUT
ARTICLE & TOPICS
Article category

Browse all articles >

Topics

Browse all articles >

BROWSE ARTICLES
AUTHOR INFORMATION
Editorial Office
101-3503, Lotte Castle President, 109 Mapo-daero, Mapo-gu, Seoul 04146, Korea
Tel: +82-2-792-5128    Fax: +82-2-792-4089    E-mail: journal@anesthesia.or.kr                

Copyright © 2021 by Korean Society of Anesthesiologists. All rights reserved.

Developed in M2PI

Close layer
prev next