An Anesthetic Experience in a Patient with Niemann-Pick Disease: A case report / 대한마취과학회지
Korean Journal of Anesthesiology
;
: 109-112, 2008.
Artículo
en 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:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Piperidinas
/
Infecciones del Sistema Respiratorio
/
Ataxia
/
Convulsiones
/
Esfingomielina Fosfodiesterasa
/
Gastrostomía
/
Trastornos de Deglución
/
Propofol
/
Enfermedades de Niemann-Pick
/
Colesterol
Límite:
Femenino
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Anesthesiology
Año:
2008
Tipo del documento:
Artículo
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