Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://doi.org/10.25751/rspa.25544 |
Resumo: | Hemophilia B is a coagulation disorder characterized by a deficiency of clotting factor IX. Women are often heterozygous carriers of the disease, however if their clotting factor levels are less than 60%, they may have an increased bleeding tendency. This is even higher if levels are under 40%. We present a case of a 14-year-old female, with mild hemophilia B (hemophilia B carrier with factor IX level < 40%) who underwent a major surgery: a posterior spinal instrumentation from D6 to L1. The perioperative management was discussed, including the perioperative administration of blood products and coagulation adjuncts. This was coordinated by a multidisciplinary team (orthopedists, anesthesiologists, hematologists and nurses) to provide the best perioperative care and follow-up. Close collaboration and communication among/with the team members and the patient/family was vital throughout. |
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Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentationAbordagem perioperatória de uma adolescente com hemofilia B submetida a instrumentação posterior da coluna dorsolombarCaso ClínicoHemophilia B is a coagulation disorder characterized by a deficiency of clotting factor IX. Women are often heterozygous carriers of the disease, however if their clotting factor levels are less than 60%, they may have an increased bleeding tendency. This is even higher if levels are under 40%. We present a case of a 14-year-old female, with mild hemophilia B (hemophilia B carrier with factor IX level < 40%) who underwent a major surgery: a posterior spinal instrumentation from D6 to L1. The perioperative management was discussed, including the perioperative administration of blood products and coagulation adjuncts. This was coordinated by a multidisciplinary team (orthopedists, anesthesiologists, hematologists and nurses) to provide the best perioperative care and follow-up. Close collaboration and communication among/with the team members and the patient/family was vital throughout.Hemofilia B é um distúrbio da coagulação caracterizado por uma deficiência do fator IX da coagulação. As mulheres são frequentemente portadoras heterozigóticas da doença, contudo se estas apresentarem níveis de fator inferiores a 60% podem ter uma maior tendência para fenómenos hemorrágicos. Esta tendência é particularmente acentuada se os níveis de fator IX forem inferiores a 40%. Apresentamos o caso de uma rapariga de 14 anos com hemofilia B ligeira (portadora de hemofilia B com nível de fator IX < 40%) que foi submetida a uma cirurgia hemorrágica (instrumentação posterior da coluna de D6 a L1). Discutimos a abordagem perioperatória, incluindo a administração perioperatória de hemoderivados. Para providenciar os melhores cuidados perioperatórios, uma equipa multidisciplinar coordenada (ortopedistas, anestesiologistas, hematologistas, enfermeiros e serviço de Imunohemoterapia) e uma boa comunicação entre os membros da equipa e o doente/família foram fundamentais.Sociedade Portuguesa de Anestesiologia2022-04-22T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25751/rspa.25544eng0871-6099Martinho, LilianaPerry da Câmara, LuisaBatalha, SaraRodrigues, JoanaCarioca, FilipaMarques, Joanainfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-23T15:34:53Zoai:ojs.revistas.rcaap.pt:article/25544Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:04:20.489831Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation Abordagem perioperatória de uma adolescente com hemofilia B submetida a instrumentação posterior da coluna dorsolombar |
title |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
spellingShingle |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation Martinho, Liliana Caso Clínico |
title_short |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
title_full |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
title_fullStr |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
title_full_unstemmed |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
title_sort |
Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation |
author |
Martinho, Liliana |
author_facet |
Martinho, Liliana Perry da Câmara, Luisa Batalha, Sara Rodrigues, Joana Carioca, Filipa Marques, Joana |
author_role |
author |
author2 |
Perry da Câmara, Luisa Batalha, Sara Rodrigues, Joana Carioca, Filipa Marques, Joana |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Martinho, Liliana Perry da Câmara, Luisa Batalha, Sara Rodrigues, Joana Carioca, Filipa Marques, Joana |
dc.subject.por.fl_str_mv |
Caso Clínico |
topic |
Caso Clínico |
description |
Hemophilia B is a coagulation disorder characterized by a deficiency of clotting factor IX. Women are often heterozygous carriers of the disease, however if their clotting factor levels are less than 60%, they may have an increased bleeding tendency. This is even higher if levels are under 40%. We present a case of a 14-year-old female, with mild hemophilia B (hemophilia B carrier with factor IX level < 40%) who underwent a major surgery: a posterior spinal instrumentation from D6 to L1. The perioperative management was discussed, including the perioperative administration of blood products and coagulation adjuncts. This was coordinated by a multidisciplinary team (orthopedists, anesthesiologists, hematologists and nurses) to provide the best perioperative care and follow-up. Close collaboration and communication among/with the team members and the patient/family was vital throughout. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-04-22T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.25751/rspa.25544 |
url |
https://doi.org/10.25751/rspa.25544 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
0871-6099 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Anestesiologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Anestesiologia |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
repository.mail.fl_str_mv |
|
_version_ |
1799130506643636225 |