Congenital factor XIII deficiency in the south of Tunisia

Pathologie-biologie
Moez MedhaffarT Souissi

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.

References

Aug 1, 1975·Australian and New Zealand Journal of Medicine·D G Woodfield, A Eastman
Jan 1, 1990·Gynecologic and Obstetric Investigation·T KobayashiH Saito
Jan 1, 1988·The American Journal of Pediatric Hematology/oncology·S L AbbondanzoR A McPherson
Feb 1, 1985·Acta Orthopaedica Scandinavica·L ClaesW Mutschler
Apr 22, 1999·Archives of Disease in Childhood. Fetal and Neonatal Edition·C J KillickG Standen
May 11, 2000·Seminars in Thrombosis and Hemostasis·A IchinoseN Takahashi
Jul 23, 2003·Journal of Thrombosis and Haemostasis : JTH·E AjznerL Muszbek
Aug 13, 2003·Journal of Thrombosis and Haemostasis : JTH·M LakP M Mannucci
Dec 17, 2003·Journal of Thrombosis and Haemostasis : JTH·I JenningsUNKNOWN UK NEQAS
Dec 26, 2003·Human Mutation·Flora PeyvandiPier Mannuccio Mannucci

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Citations

Apr 3, 2012·Annales Françaises D'anesthèsie Et De Rèanimation·A El KoraichiS E El Kettani
Sep 3, 2013·Haemophilia : the Official Journal of the World Federation of Hemophilia·L A T Sharief, R A Kadir
Jul 14, 2017·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Ines MaaloulMongia Hachicha

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