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Korean Journal of Anesthesiology 2008;54(1):109-112.
DOI: https://doi.org/10.4097/kjae.2008.54.1.109   
An Anesthetic Experience in a Patient with Niemann-Pick Disease: A case report.
Jun Hwa Lee, Woo Suk Jung, Ho Yung Lee, A Reum Kim, Hee Suk Yoon, Yung Kwon Go
Department of Anesthesiology and Pain Medicine, College of Medicine, Chungnam University, Daejeon, Korea. meister@cnu.ac.kr
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.
Key Words: endotracheal intubation; Niemann-Pick disease; propofol; remifentanil


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