2017
DOI: 10.11604/pamj.2017.27.147.12248
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Von Willebrand's disease: case report and review of literature

Abstract: Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD us… Show more

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Cited by 24 publications
(31 citation statements)
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“…La prevalencia del tipo 3 es de 1 a 5 por millón de habitantes en Europa, y de 3 por millón en Suecia e Israel (13). En Chile y Venezuela representan el 2,71, 1,75 por millón, respectivamente (14).…”
Section: Epidemiologíaunclassified
See 1 more Smart Citation
“…La prevalencia del tipo 3 es de 1 a 5 por millón de habitantes en Europa, y de 3 por millón en Suecia e Israel (13). En Chile y Venezuela representan el 2,71, 1,75 por millón, respectivamente (14).…”
Section: Epidemiologíaunclassified
“…El FVW es sintetizado por células endoteliales y megacariocitos (20,23), almacenado en gránulos alfa y cuerpos de Weibel-Palade (23). Además, es modulado y clivado por las metaloproteinasas ADAMTS 13 al momento de entrar al torrente sanguíneo (14,20,24). La estructura de esta proteína comprende dominios de la A a la D, estos últimos involucrados en la regulación de la formación multimérica y su adhesión al FVIII de la coagulación.…”
Section: Estructura Molecular Del Factor De Von Willebrandunclassified
“…Фактор XII необходим для образования фибрина in vitro. Однако у пациентов с дефицитом фактора XII не наблюдаются клинически значимые кровотечения, несмотря на удлинение АЧТВ [55]. Фактически некоторые исследования подтверждают положение о связи между дефицитом фактора XII и повышенным риском тромбоза, возможно, из-за роли фактора XII в фибринолизе [2].…”
Section: фактор XIIunclassified
“…Background vWD is a common IBD affecting 1.3% of the population. [5,6] It is caused by a quantitative (reduced amount) or qualitative (abnormal function) deficiency in von Willebrand factor (vWF), combinations of which result in the various vWD subtypes (Table 3). vWF is synthesised by endothelial cells and megakaryocytes and is a complex…”
Section: Von Willebrand Diseasementioning
confidence: 99%
“…Patients with vWD types 2A, 2B and 3 usually require replacement therapy post-partum to prevent immediate or delayed haemorrhage. [5][6][7][8]…”
Section: Clinical Presentationmentioning
confidence: 99%