Surgical treatment of ruptured ovarian cyst in a patient with Bernard-Soulier syndrome

Introduction: the Bernard-Soulier Syndrome (BSS) is a rare inherited bleeding disorder witch the prevalence is estimated at 1:1,000,000. Its abnormalities are due to defective expression of the glycoproteic complex GPIb/IX/V – the platelets receptor that is crucial for the establishment of the prime...

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Detalles Bibliográficos
Autores: Pires, João Victor Alcântara, Santana, Julliana Ferrari Campêlo Libório de, Fernandes, Etienny de Brito Dias, Berton, Nadia Cristina, Franchello, Isabela Furlan, Santos, Rubia Martinez, Marques, Camila Paixão, Francisco, Letícia Cristino, Lara, Renata Fornaciari
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:Brasil
Institución:Universidade Federal de Itajubá (UNIFEI)
Repositorio:Research, Society and Development
Idioma:portugués
OAI Identifier:oai:ojs.pkp.sfu.ca:article/25366
Acceso en línea:https://rsdjournal.org/index.php/rsd/article/view/25366
Access Level:acceso abierto
Palabra clave:Quiestes Ováricos
Síndrome de Bernard-Soulier
Abdomen agudo.
Ovarian Cysts
Bernard-Soulier Syndrome
Abdomen acute.
Cistos Ovarianos
Abdome agudo.
Descripción
Sumario:Introduction: the Bernard-Soulier Syndrome (BSS) is a rare inherited bleeding disorder witch the prevalence is estimated at 1:1,000,000. Its abnormalities are due to defective expression of the glycoproteic complex GPIb/IX/V – the platelets receptor that is crucial for the establishment of the prime hemostasis. Objective: report a rare case of a ruptured ovarian cyst in a patient with Bernard-Soulier Síndromes. Methodology: it's a descriptive and transversal study that consists of the formulation of a case report, built from the analysis of a patient’s medical record with BSS, who evolved with ruptured ovarian cyst. For theoretical foundation we used articles indexed in PubMed's database written in portuguese and english. The following descriptors were considered: Ovarian Cysts, Bernard-Soulier Syndrome and Abdomen Acute. Conclusion: considering the frequency and severity of the hemorrhagic events as highly variable aspects among patients with BSS it is necessary that the surgeon always keep in mind the underlying hematological disorders when faced with a possible hemorrhagic acute abdomen.

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