[Congenital factor XIII deficiency in the south of Tunisia].
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.
Medhaffar
M
M
Service d'hématologie, hôpital Hédi-Chaker, route El-Ain 3029, Sfax, Tunisie. mmmoez11df@yahoo.fr
Elloumi
M
M
Guermazi
S
S
Kallel
C
C
Mseddi
S
S
Bellaaj
H
H
Makni
F
F
Souissi
T
T
D005177:Factor XIII Deficiency / Q000151:congenital* / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D014416:Tunisia