Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report
Autor(a) principal: | |
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Data de Publicação: | 2021 |
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/10.25754/pjp.2021.20470 |
Resumo: | Hemophilia A, characterized by deficiency of functional plasma clotting factor VIII, is an X-linked disorder. Signs and symptoms depend on factor VIII levels. Severe hemophilia A (factor levels less than 1%) is usually diagnosed in the first two years of life. Pediatric compartment syndrome is most associated with trauma, but infectious and vascular causes must also be considered. We report a case of a newborn who presented with a severe hand hematoma after venous puncture, complicated by compartment syndrome. An isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemophilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure. |
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Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case ReportCase reportsHemophilia A, characterized by deficiency of functional plasma clotting factor VIII, is an X-linked disorder. Signs and symptoms depend on factor VIII levels. Severe hemophilia A (factor levels less than 1%) is usually diagnosed in the first two years of life. Pediatric compartment syndrome is most associated with trauma, but infectious and vascular causes must also be considered. We report a case of a newborn who presented with a severe hand hematoma after venous puncture, complicated by compartment syndrome. An isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemophilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure.A Hemofilia A é uma doença ligada ao X caracterizada pelo défice de factor de coagulação VIII (FVIII) no plasma. Os sinais e sintomas correlacionam-se com os níveis de factor VIII. A Hemofilia A grave, com níveis de factor inferiores a 1%, é geralmente diagnosticada nos primeiros dois anos de vida. A síndrome compartimental em idade pediátrica ocorre habitualmente em contexto de trauma, mas as etiologias vascular e infeciosa devem ser consideradas. Descrevemos o caso de um recém-nascido com hematoma exuberante da mão complicado de síndrome compartimental, após punção venosa. Na avaliação laboratorial realizada destaca-se o prolongamento isolado do Tempo de Tromboplastina Parcial activado, tendo sido assumido o diagnóstico de Hemofilia, confirmado após doseamento de FVIII < 1% (FIX normal). Após a administração de factor VIII recombinante foi realizada fasciotomia; a antibioterapia endovenosa foi mantida até ao encerramento da fasciotomia. Sociedade Portuguesa de Pediatria2021-07-16info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25754/pjp.2021.20470eng2184-44532184-3333Sacras, Maria LuísLadeira, CatarinaCarmo, SaraKjöllerström, Paulainfo: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:RCAAP2023-08-03T02:58:11Zoai:ojs.revistas.rcaap.pt:article/20470Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:25:35.580092Repositório 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 |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
title |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
spellingShingle |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report Sacras, Maria Luís Case reports |
title_short |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
title_full |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
title_fullStr |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
title_full_unstemmed |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
title_sort |
Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report |
author |
Sacras, Maria Luís |
author_facet |
Sacras, Maria Luís Ladeira, Catarina Carmo, Sara Kjöllerström, Paula |
author_role |
author |
author2 |
Ladeira, Catarina Carmo, Sara Kjöllerström, Paula |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Sacras, Maria Luís Ladeira, Catarina Carmo, Sara Kjöllerström, Paula |
dc.subject.por.fl_str_mv |
Case reports |
topic |
Case reports |
description |
Hemophilia A, characterized by deficiency of functional plasma clotting factor VIII, is an X-linked disorder. Signs and symptoms depend on factor VIII levels. Severe hemophilia A (factor levels less than 1%) is usually diagnosed in the first two years of life. Pediatric compartment syndrome is most associated with trauma, but infectious and vascular causes must also be considered. We report a case of a newborn who presented with a severe hand hematoma after venous puncture, complicated by compartment syndrome. An isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemophilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-07-16 |
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/10.25754/pjp.2021.20470 |
url |
https://doi.org/10.25754/pjp.2021.20470 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
2184-4453 2184-3333 |
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 Pediatria |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Pediatria |
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) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799133524858503168 |