General anesthesia for Crisponi syndrome: case report
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , , , |
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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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 |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0034-70942020000300299 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0034-70942020000300299 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.bjane.2020.04.010 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
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) instacron:SBA |
instname_str |
Sociedade Brasileira de Anestesiologia (SBA) |
instacron_str |
SBA |
institution |
SBA |
reponame_str |
Revista Brasileira de Anestesiologia (Online) |
collection |
Revista Brasileira de Anestesiologia (Online) |
repository.name.fl_str_mv |
Revista Brasileira de Anestesiologia (Online) - Sociedade Brasileira de Anestesiologia (SBA) |
repository.mail.fl_str_mv |
||sba2000@openlink.com.br |
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1752126630801702912 |