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Variant Bernard-Soulier syndrome type bolzano. A congenital bleeding disorder due to a structural and functional abnormality of the platelet glycoprotein Ib-IX complex.

We have studied a patient with a congenital bleeding disorder and phenotypic manifestations typical of Bernard-Soulier syndrome, including giant platelets with absent ristocetin-induced von Willebrand factor binding. Two monoclonal antibodies reacting with distinct epitopes in the amino-terminal dom...

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Detalles Bibliográficos
Publicado en:J Clin Invest
Principais autores: De Marco, L, Mazzucato, M, Fabris, F, De Roia, D, Coser, P, Girolami, A, Vicente, V, Ruggeri, Z M
Formato: Artigo
Idioma:Inglês
Publicado: American Society for Clinical Investigation 1990
Assuntos:
Acceso en liña:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC296685/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/1694864/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI114692
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