Congenital factor XIII deficiency associated with von willebrand disease | Zendy

Grand B. | Zendy; Blanco A. | Zendy; Riveros D. | Zendy; Ventura A. | Zendy; Maugeri N. | Zendy; Woods A. | Zendy; Lazzari M. | Zendy

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Congenital factor XIII deficiency associated with von willebrand disease

Author(s) -

Grand B.,

Blanco A.,

Riveros D.,

Ventura A.,

Maugeri N.,

Woods A.,

Lazzari M.

Publication year - 1990

Publication title -

american journal of hematology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.456

H-Index - 105

eISSN - 1096-8652

pISSN - 0361-8609

DOI - 10.1002/ajh.2830350313

Subject(s) - von willebrand disease , medicine , von willebrand factor , factor xiii deficiency , complication , coagulopathy , coagulation , factor xiii , heterozygote advantage , gastroenterology , surgery , pediatrics , fibrinogen , platelet , allele , biochemistry , chemistry , gene

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.

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