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Anesthetic Management in a Child With Niemann-Pick Disease
Espahbodi, Ebrahim; Yaghooti, Amir Abbas; Ostadalipour, Abbas & Marashi, Shaqayeq
Abstract
Niemann-Pick is a lipid storage disease that results from a lysosomal enzyme deficiency (sphingomyelinase). It has different presentations,
and it may affect various organs such as the central nervous system, kidney, liver, and spleen. Due to the complexity of the
disease, careful perianesthetic management is necessary in order to reduce the risks and sequels. As there is little evidence available
in the literature regarding the anesthetic implications of such patients, in this case report we describe the anesthetic management
of a two-year-old female with Niemann-Pick disease.
Keywords
Niemann-Pick; Anesthesia; Child
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