General anesthesia for Crisponi syndrome: case report

Detalhes bibliográficos
Autor(a) principal: Allary,Chloé
Data de Publicação: 2020
Outros Autores: Caruselli,Marco, Fabre,Alexandre, Audic,Frédérique, Michel,Fabrice
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Revista Brasileira de Anestesiologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0034-70942020000300299
Resumo: Abstract Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19ºC. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.
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spelling General anesthesia for Crisponi syndrome: case reportCrisponi syndromePediatric anesthesiaSudden deathBody temperature regulationMalignant hyperthermiaAbstract Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19ºC. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.Sociedade Brasileira de Anestesiologia2020-06-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0034-70942020000300299Revista Brasileira de Anestesiologia v.70 n.3 2020reponame:Revista Brasileira de Anestesiologia (Online)instname:Sociedade Brasileira de Anestesiologia (SBA)instacron:SBA10.1016/j.bjane.2020.04.010info:eu-repo/semantics/openAccessAllary,ChloéCaruselli,MarcoFabre,AlexandreAudic,FrédériqueMichel,Fabriceeng2020-09-09T00:00:00Zoai:scielo:S0034-70942020000300299Revistahttps://www.sbahq.org/revista/https://old.scielo.br/oai/scielo-oai.php||sba2000@openlink.com.br1806-907X0034-7094opendoar:2020-09-09T00:00Revista Brasileira de Anestesiologia (Online) - Sociedade Brasileira de Anestesiologia (SBA)false
dc.title.none.fl_str_mv General anesthesia for Crisponi syndrome: case report
title General anesthesia for Crisponi syndrome: case report
spellingShingle General anesthesia for Crisponi syndrome: case report
Allary,Chloé
Crisponi syndrome
Pediatric anesthesia
Sudden death
Body temperature regulation
Malignant hyperthermia
title_short General anesthesia for Crisponi syndrome: case report
title_full General anesthesia for Crisponi syndrome: case report
title_fullStr General anesthesia for Crisponi syndrome: case report
title_full_unstemmed General anesthesia for Crisponi syndrome: case report
title_sort General anesthesia for Crisponi syndrome: case report
author Allary,Chloé
author_facet Allary,Chloé
Caruselli,Marco
Fabre,Alexandre
Audic,Frédérique
Michel,Fabrice
author_role author
author2 Caruselli,Marco
Fabre,Alexandre
Audic,Frédérique
Michel,Fabrice
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Allary,Chloé
Caruselli,Marco
Fabre,Alexandre
Audic,Frédérique
Michel,Fabrice
dc.subject.por.fl_str_mv Crisponi syndrome
Pediatric anesthesia
Sudden death
Body temperature regulation
Malignant hyperthermia
topic Crisponi syndrome
Pediatric anesthesia
Sudden death
Body temperature regulation
Malignant hyperthermia
description Abstract Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19ºC. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.
publishDate 2020
dc.date.none.fl_str_mv 2020-06-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0034-70942020000300299
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dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1016/j.bjane.2020.04.010
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dc.publisher.none.fl_str_mv Sociedade Brasileira de Anestesiologia
publisher.none.fl_str_mv Sociedade Brasileira de Anestesiologia
dc.source.none.fl_str_mv Revista Brasileira de Anestesiologia v.70 n.3 2020
reponame:Revista Brasileira de Anestesiologia (Online)
instname:Sociedade Brasileira de Anestesiologia (SBA)
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collection Revista Brasileira de Anestesiologia (Online)
repository.name.fl_str_mv Revista Brasileira de Anestesiologia (Online) - Sociedade Brasileira de Anestesiologia (SBA)
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