Singularities of haemorrhagic syndrome in children with native and acquired deficiencies of von Willebrand factor


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Abstract

Deficiencies of content in blood and activity of von Willebrand factor can be inborn or acquired with diseases of different nature. Acquired deficiencies of von Willebrand factor or acquired von Willebrand syndrome in children have been described in several clinical studies. This research paper contains data on clinical manifestation and dynamics of haemorrhagic syndrome in 30 children between 13 months and 18 years of age with acquired von Willebrand factor, suffering from different types of pathology. Similarly, clinical manifestations and dynamics of angiostaxis have been studied in 33 children with von Willebrand disease. It has been determined that clinical manifestations of microcirculatory angiostaxis are the same for children from both groups; however, children with acquired von Willebrand syndrome showed dominating limited numbers of haemorrhagical symptoms that were shorter in duration and less intense. Besides, hematomic component of haemorrhagic syndrome was absent. Eventually, accompanied by positive dynamic of the underlying disease, relapses of haemorrhagic syndrome cease, which does not happen in case of the patients with von Willebrand disease.

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About the authors

Kseniya Ivanovna Pshenichnaya

Saint-Petersburg State Pediatric Medical University

Email: kpshenichnaya@yandex.ru
MD, Pediatrician, Hematologist, Professor of Pediatrics Department

Yegor Viktorovich Lyugayev

Saint-Petersburg State Pediatric Medical University

Email: kpshenichnaya@yandex.ru
Postgraduate Student, Pediatrics Department, Pediatrician

Olga Georgiyevna Golovina

Russian Research Institute of Hematology and Blood Transfusion

Email: olga.golovina.48@mail.ru
Ph.D., Senior Researcher

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Copyright (c) 2013 Pshenichnaya K.I., Lyugayev Y.V., Golovina O.G.

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