An Anesthetic Experience in a Patient with Niemann-Pick Disease: A case report / 대한마취과학회지
Korean Journal of Anesthesiology
;
: 109-112, 2008.
Artigo
em Coreano
| WPRIM
| ID: wpr-181755
ABSTRACT
Niemann-Pick disease (NPD) is an autosomal recessive, lipid storage disorder caused by the deficiency of the lysosomal enzyme sphingomyelinase or defective cholesterol transport from lysosome to cytosol. The clinical symptoms and signs include dysphagia, loss of motor function, hepatosplenomegaly, recurred respiratory infections, seizure, mental retardation, spasticity, myoclonic jerks and ataxia, but vary depending on the type of this disease. We report a successful anesthetic experience, including endotracheal intubation with Glidescope under propofol and remifentanil infusion without neuromuscular blockade, in a 21-year old woman with Niemann-Pick disease for wound revision of gastrostomy site.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Piperidinas
/
Infecções Respiratórias
/
Ataxia
/
Convulsões
/
Esfingomielina Fosfodiesterase
/
Gastrostomia
/
Transtornos de Deglutição
/
Propofol
/
Doenças de Niemann-Pick
/
Colesterol
Limite:
Feminino
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Anesthesiology
Ano de publicação:
2008
Tipo de documento:
Artigo
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