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Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature

BACKGROUND: Mitochondria are the energy producing organelles practically in every human cell except erythrocytes. Indeed mitochondria are widespread in high energy requiring organs like brain, heart and muscles. Currently there are no clinical trials supporting with clear evidence which is the most...

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Autores principales: Lilitsis, Emmanuel, Astyrakaki, Elisavet, Blevrakis, Evaggelos, Xenaki, Sofia, Chalkiadakis, George, Chrysos, Emmanuel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5576333/
https://www.ncbi.nlm.nih.gov/pubmed/28851284
http://dx.doi.org/10.1186/s12871-017-0400-9
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author Lilitsis, Emmanuel
Astyrakaki, Elisavet
Blevrakis, Evaggelos
Xenaki, Sofia
Chalkiadakis, George
Chrysos, Emmanuel
author_facet Lilitsis, Emmanuel
Astyrakaki, Elisavet
Blevrakis, Evaggelos
Xenaki, Sofia
Chalkiadakis, George
Chrysos, Emmanuel
author_sort Lilitsis, Emmanuel
collection PubMed
description BACKGROUND: Mitochondria are the energy producing organelles practically in every human cell except erythrocytes. Indeed mitochondria are widespread in high energy requiring organs like brain, heart and muscles. Currently there are no clinical trials supporting with clear evidence which is the most suitable surgical or anesthetic management of a patient with known mitochondrial disease presenting with surgical disorders. This condition poses possible hazardous problems to the medical attention of those patients. CASE PRESENTATION: A case of an 8 year old child with known Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH deficiency) requiring surgery for acute appendicitis is presented. Our approach for anesthesia revealed a combination of fentanyl, low dose propofol and nitrous oxide. CONCLUSION: The choice of the safest pharmacological anesthetic agents for patients with ETFDH deficiency is challenging given that most of the general anesthetic medications have multiple effects on mitochondria, fatty acids metabolism and striated muscles. Anesthetists are expected to individualize anesthetic care for the patient based on current publications for similar cases, medical history and knowledge of pharmacology and physiology.
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spelling pubmed-55763332017-08-31 Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature Lilitsis, Emmanuel Astyrakaki, Elisavet Blevrakis, Evaggelos Xenaki, Sofia Chalkiadakis, George Chrysos, Emmanuel BMC Anesthesiol Case Report BACKGROUND: Mitochondria are the energy producing organelles practically in every human cell except erythrocytes. Indeed mitochondria are widespread in high energy requiring organs like brain, heart and muscles. Currently there are no clinical trials supporting with clear evidence which is the most suitable surgical or anesthetic management of a patient with known mitochondrial disease presenting with surgical disorders. This condition poses possible hazardous problems to the medical attention of those patients. CASE PRESENTATION: A case of an 8 year old child with known Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH deficiency) requiring surgery for acute appendicitis is presented. Our approach for anesthesia revealed a combination of fentanyl, low dose propofol and nitrous oxide. CONCLUSION: The choice of the safest pharmacological anesthetic agents for patients with ETFDH deficiency is challenging given that most of the general anesthetic medications have multiple effects on mitochondria, fatty acids metabolism and striated muscles. Anesthetists are expected to individualize anesthetic care for the patient based on current publications for similar cases, medical history and knowledge of pharmacology and physiology. BioMed Central 2017-08-29 /pmc/articles/PMC5576333/ /pubmed/28851284 http://dx.doi.org/10.1186/s12871-017-0400-9 Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Lilitsis, Emmanuel
Astyrakaki, Elisavet
Blevrakis, Evaggelos
Xenaki, Sofia
Chalkiadakis, George
Chrysos, Emmanuel
Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title_full Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title_fullStr Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title_full_unstemmed Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title_short Anesthetic management of a pediatric patient with Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH) and acute appendicitis: case report and review of the literature
title_sort anesthetic management of a pediatric patient with electron transfer flavoprotein dehydrogenase deficiency (etfdh) and acute appendicitis: case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5576333/
https://www.ncbi.nlm.nih.gov/pubmed/28851284
http://dx.doi.org/10.1186/s12871-017-0400-9
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