title: Congenital factor XIII deficiency associated with von Willebrand disease.
abstract: A boy with umbilical bleeding and severe hemorrhages after minor trauma, without family bleeding history, was studied. Coagulation tests showed abnormalities in FXIII subunits and FVIII/vWF complex. Both parents presented results compatible with a heterozygote state for FXIII deficiency and the father had abnormalities of FVIII/vWF. The propositus was diagnosed as congenital FXIII deficiency associated with vWD. No severe hemorrhagic complication was observed after a prophylactic regimen with cryoprecipitates.
authors.affiliation: Department of hemostasis and Thrombosis, National Academy of Medicine, Buenos Aires, Argentina.
authors.forename: B
authors.initials: B
authors.lastname: Grand
authors.forename: A
authors.initials: A
authors.lastname: Blanco
authors.forename: D
authors.initials: D
authors.lastname: Riveros
authors.forename: A
authors.initials: A
authors.lastname: Ventura
authors.forename: N
authors.initials: N
authors.lastname: Maugeri
authors.forename: A
authors.initials: A
authors.lastname: Woods
authors.forename: M
authors.initials: M
authors.lastname: Lazzari
mesh_terms: D005177:Factor XIII Deficiency / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis