General Anesthesia in a Patient with Infantile Pompe's Disease : A case report
Anesthesia and Pain Medicine
;
: 124-128, 2006.
Artigo
em Coreano
| WPRIM
| ID: wpr-81787
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Fenômenos Fisiológicos Respiratórios
/
Traqueostomia
/
Doença de Depósito de Glicogênio Tipo II
/
Ventiladores Mecânicos
/
Músculo Esquelético
/
Terapia de Reposição de Enzimas
/
Glucosidases
/
Glicogênio
/
Anestesia
/
Anestesia Geral
Limite:
Humanos
/
Lactente
Idioma:
Coreano
Revista:
Anesthesia and Pain Medicine
Ano de publicação:
2006
Tipo de documento:
Artigo
Similares
MEDLINE
...
LILACS
LIS