Paper Information

Journal:   JOURNAL OF ISFAHAN MEDICAL SCHOOL (I.U.M.S)   1ST WEEK, FEBRUARY 2014 , Volume 31 , Number 263; Page(s) 1985 To 1993.
 
Paper: 

CENTRAL NERVOUS SYSTEM BLEEDING IN FACTOR XIII DEFICIENCY, ASSESSMENT THE ROLE OF PLASMINOGEN ACTIVATOR INHIBITOR-1

 
 
Author(s):  NADERI MAJID, DORGALALEH AKBAR, SHABAN ALIZADEH*, KAZEMI AHMAD, TABIBIAN SHADI, GHUTASLOU ABBAS, KASHIRI MEYSAM, HOSSEINI HOSNIEH, HOSSEINI MARYAM AL SADAT
 
* DEPARTMENT OF HEMATOLOGY, ALLIED MEDICAL SCHOOL, TEHRAN UNIVERSITY OF MEDICAL SCIENCES, TEHRAN, IRAN
 
Abstract: 

Background: Factor XIII deficiency is a rare bleeding disorder (RBD) with a high incidence in Sistan and Baluchistan province, Iran. Central nervous system (CNS) bleeding is a common but lifethreatening clinical presentation of severe factor XIII deficiency. This study aimed to assess the role of PAI-14G/5G polymorphism in occurrence of intra and extracranial hemorrhage in factor 13 deficiency.
Methods: In this study, 64 patient with factor XIII deficiency were enrolled. Initially, according to the history of occurrence of CNS bleeding, patients were divided in two groups of case and control. At the baseline, both groups were evaluated for the Trp187Arg polymorphism in order to confirm their disorder. Then, all patients were assessed for PAI-14G/5G polymorphism.
Findings: All study patients were homozygote for factor XIII polymorphism. We also found that the equal numbers of patients (4 individuals) in case and control groups were heterozygote for PAI-14G/5G polymorphism and none of patients were homozygote for this polymorphism. All heterozygote patients had intracranial hemorrhage and patients with extracranial hemorrhage had no mutation of PAI-14G/5G.
Conclusion: PAI-14G/5G polymorphism did not any effect on occurrence of intra and extracranial hemorrhage in patients with factor 13 deficiency.

 
Keyword(s): F13 DEFICIENCY, CENTRAL NERVOUS SYSTEM (CNS) BLEEDING, INTRACRANIAL HEMORRHAGE, EXTRACRANIAL HEMORRHAGE
 
References: 
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