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2019, Number 5-6

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Rev Sanid Milit Mex 2019; 73 (5-6)

Von Willebrand disease: clinical signs, diagnosis and treatment

Palmero‑Picazo J, Rodríguez‑Gallegos MF

Language: Spanish
References: 40
Page: 282-287
PDF size: 227.80 Kb.


ABSTRACT

Von Willebrand disease is the most common hereditary hemorrhagic disorder, characterized by a tendency to hemorrhage due to a quantitative or qualitative alteration of the Von Willebrand factor. Objectives: To present the clinical picture of Von Willebrand’s disease, its etiology, physiopathology, as well as the diagnostic integration and updated treatment. Material and methods: Descriptive observational study carried out through the systematic search of EBSCO and PUBMED, using key words such as: Von Willebrand disease, Von Willebrand factor, coagulopathy, phenotype, genotype; selecting arbitrarily the most relevant articles in English, after the year 2015. 40 articles were obtained. Results: There are three types: 1, 2A, 2B, 2N, 2M, and 3. Within its etiopathogenesis, an association with mutations and genetic alterations is shown, mainly on chromosome 12, in the p13.2 region, whose function is possess the code that will serve for the transcription and translation of the Von Willebrand factor. Its diagnosis is through directed interrogation, coagulation studies and the determination of Von Willegrand’s multimers. The treatment is based on desmopressin, vWF concentrates, antifibrinolytics, estrogens and platelet transfusions. Conclusions: The diagnosis and adequate treatment is essential to avoid complications and fatal hemorrhages, mainly during surgery or odontological procedures.


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