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Endodontic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome is a rare inherited disorder with giant platelets, thrombocytopenia and a prolonged bleeding time. These abnormalities are caused by genetic defects of the glycoprotein Ib/IX/V complex that constitutes the von Willebrand factor receptor on the platelet surface. We are docume...

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Detalles Bibliográficos
Autores principales: Johns, Dexton Antony, Gopalan, Reji P, Kamble, Ganesh Tukaram, Vidyanath, S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4001280/
https://www.ncbi.nlm.nih.gov/pubmed/24778520
http://dx.doi.org/10.4103/0972-0707.128044
Descripción
Sumario:Bernard-Soulier syndrome is a rare inherited disorder with giant platelets, thrombocytopenia and a prolonged bleeding time. These abnormalities are caused by genetic defects of the glycoprotein Ib/IX/V complex that constitutes the von Willebrand factor receptor on the platelet surface. We are documenting a 30-year-old female patient reported with a chief complaint of swelling in relation to right maxillary canine for 5 days. The primary treatment remains platelet transfusion. Root canal treatment was performed following platelet rich plasma transfusion. As the tooth was necrotic and had a periapical pathology post-operative bleeding was absent. The root canal treatment was uneventful and the patient was asymptomatic. Optimum oral hygiene practices were emphasized to avoid dental diseases.