Literature DB >> 24237226

Congenital platelet disorder and type I von Willebrand disease presenting as prolonged bleeding after cleft lip and palate repair.

John Pang, Jonathan Zelken, Amir H Dorafshar, John J Strouse, Richard Redett.   

Abstract

Cleft lip and palate can be associated with coagulopathy. Here, we report the first known case of congenital platelet disorder and von Willebrand disease presenting as prolonged bleeding after cleft lip and palate repair. After identifying the underlying pathology, platelet infusions and aminocaproic acid were given to decrease bleeding from a second surgical procedure. Whole exome sequencing identified a von Willebrand factor gene mutation, an adenine to guanine substitution at the c.475A location. A high index of suspicion should be had for coagulopathy in patients with syndromic cleft lip and palate.

Entities:  

Keywords:  cleft lip; cleft palate; platelet abnormality; von Willebrand disease

Mesh:

Year:  2013        PMID: 24237226     DOI: 10.1597/13-186

Source DB:  PubMed          Journal:  Cleft Palate Craniofac J        ISSN: 1055-6656


  1 in total

1.  Von-Willebrand disease presenting as intractable epistaxis after nasal polypectomy.

Authors:  Jeong Jin Park; Chang-Hoon Kim; Jeung-Gweon Lee; Hyung-Ju Cho
Journal:  Case Rep Otolaryngol       Date:  2014-08-25
  1 in total

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