Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report

Detalhes bibliográficos
Autor(a) principal: Sacras, Maria Luís
Data de Publicação: 2021
Outros Autores: Ladeira, Catarina, Carmo, Sara, Kjöllerström, Paula
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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spelling 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
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dc.language.iso.fl_str_mv eng
language eng
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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)
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