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KMID : 0356920080540010109
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Korean Journal of Anesthesiology
2008 Volume.54 No. 1 p.109 ~ p.112
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An Anesthetic Experience in a Patient with Niemann-Pick Disease
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Lee Joon-hwa
Chung Woo-Suk
Lee Ho-Yung
Kim A-Reum
Yoon Hee-Suk
Ko Young-Kwon
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Abstract
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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. (Korean J Anesthesiol 2008; 54: 109∼12)
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KEYWORD
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endotracheal intubation, Niemann-Pick disease, propofol, remifentanil
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