Literature DB >> 27468167

Management of type 2b von Willebrand disease in the neonatal period.

Lindsay Proud1, A Kim Ritchey2.   

Abstract

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting one in 1,000 people. Type 2b VWD is a less common subtype caused by a gain-of-function mutation in von Willebrand factor (VWF) that leads to the formation of large, ineffective VWF-platelet multimers in circulation. This unique pathophysiology creates diagnostic and treatment dilemmas. There is limited information on the management of type 2b VWD in the neonatal period. This report describes the management of a neonate with type 2b VWD with an emphasis on the added benefit of concomitant platelet transfusion and factor replacement therapy over factor replacement therapy alone.
© 2016 Wiley Periodicals, Inc.

Entities:  

Keywords:  management; neonatal; platelet transfusion; von Willebrand disease

Mesh:

Substances:

Year:  2016        PMID: 27468167     DOI: 10.1002/pbc.26168

Source DB:  PubMed          Journal:  Pediatr Blood Cancer        ISSN: 1545-5009            Impact factor:   3.167


  3 in total

1.  Protein kinase C signaling dysfunction in von Willebrand disease (p.V1316M) type 2B platelets.

Authors:  Caterina Casari; David S Paul; Sophie Susen; Cécile Lavenu-Bombled; Annie Harroche; Raymond Piatt; Kathryn O Poe; Robert H Lee; Marijke Bryckaert; Olivier D Christophe; Peter J Lenting; Cécile V Denis; Wolfgang Bergmeier
Journal:  Blood Adv       Date:  2018-06-26

2.  Case Report: An Infant with Severe Thrombocytopenia Diagnosed with Type 2B von Willebrand Disease Due To a De Novo p.Val1316Met Mutation

Authors:  Junjie Fan; Jing Ling; Huifeng Zhou; Jie He; Shaoyan Hu
Journal:  Turk J Haematol       Date:  2020-07-03       Impact factor: 1.831

3.  Management of pregnancy and emergency caesarean delivery in a patient with type IIB von Willebrand disease and severe preeclampsia: A case report and literature review.

Authors:  Daniel Martingano; Xin Guan; Francis X Martingano
Journal:  Obstet Med       Date:  2017-09-13
  3 in total

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