Anesthetic Management in a Child With Niemann-Pick Disease

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 man...

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Autores principales: Espahbodi, Ebrahim, Yaghooti, Amir Abbas, Ostadalipour, Abbas, Marashi, Shaqayeq
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Kowsar 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5294925/
https://www.ncbi.nlm.nih.gov/pubmed/28203333
http://dx.doi.org/10.5812/ijp.5479
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author Espahbodi, Ebrahim
Yaghooti, Amir Abbas
Ostadalipour, Abbas
Marashi, Shaqayeq
author_facet Espahbodi, Ebrahim
Yaghooti, Amir Abbas
Ostadalipour, Abbas
Marashi, Shaqayeq
author_sort Espahbodi, Ebrahim
collection PubMed
description 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.
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spelling pubmed-52949252017-02-15 Anesthetic Management in a Child With Niemann-Pick Disease Espahbodi, Ebrahim Yaghooti, Amir Abbas Ostadalipour, Abbas Marashi, Shaqayeq Iran J Pediatr Case Report 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. Kowsar 2016-07-31 /pmc/articles/PMC5294925/ /pubmed/28203333 http://dx.doi.org/10.5812/ijp.5479 Text en Copyright © 2016, Growth & Development Research Center http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.
spellingShingle Case Report
Espahbodi, Ebrahim
Yaghooti, Amir Abbas
Ostadalipour, Abbas
Marashi, Shaqayeq
Anesthetic Management in a Child With Niemann-Pick Disease
title Anesthetic Management in a Child With Niemann-Pick Disease
title_full Anesthetic Management in a Child With Niemann-Pick Disease
title_fullStr Anesthetic Management in a Child With Niemann-Pick Disease
title_full_unstemmed Anesthetic Management in a Child With Niemann-Pick Disease
title_short Anesthetic Management in a Child With Niemann-Pick Disease
title_sort anesthetic management in a child with niemann-pick disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5294925/
https://www.ncbi.nlm.nih.gov/pubmed/28203333
http://dx.doi.org/10.5812/ijp.5479
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