title: [Congenital factor XIII deficiency in the south of Tunisia].
abstract: Factor XIII deficiency is a rare autosomal recessive congenital disorder of haemostasis characterised by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. We report a study about ten patients with congenital factor XIII deficiency from seven south-Tunisian families, there are seven females. Umbilical bleeding was common and only two patients had intracranial bleeding. The standard screening tests are normal. Factor XIII activity was less than 1% in all patients. A sub-unit A deficit was detected for the ten patients. Out hemorrhagic context, five patients receive regular prophylactic transfusion with fresh frozen plasma.
authors.affiliation: Service d'hématologie, hôpital Hédi-Chaker, route El-Ain 3029, Sfax, Tunisie. mmmoez11df@yahoo.fr
authors.forename: M
authors.initials: M
authors.lastname: Medhaffar
authors.forename: M
authors.initials: M
authors.lastname: Elloumi
authors.forename: S
authors.initials: S
authors.lastname: Guermazi
authors.forename: C
authors.initials: C
authors.lastname: Kallel
authors.forename: S
authors.initials: S
authors.lastname: Mseddi
authors.forename: H
authors.initials: H
authors.lastname: Bellaaj
authors.forename: F
authors.initials: F
authors.lastname: Makni
authors.forename: T
authors.initials: T
authors.lastname: Souissi
mesh_terms: D005177:Factor XIII Deficiency / Q000151:congenital* / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D014416:Tunisia