Journal of Isfahan Medical School

Journal of Isfahan Medical School

Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1

Document Type : Original Article (s)

Authors
Majid Naderi 1
Akbar Dorgalaleh 2
Shaban Alizadeh 3
Ahmad Kazemi 4
Shadi Tabibian 1
Abbas Ghutaslou 2
Meysam Kashiri 2
Hosnieh Hosseini 5
Maryam-Sadat Hosseini 2
1 Associate Professor, Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, Iran
2 Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
3 Assistant Professor, Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
4 Professor, Department of Hematology, Allied Medical School, Iran University of Medical Sciences, Tehran, Iran
5 Department of Laboratory Sciences, School of Medical Sciences, Islamic Azad University, Zahedan Branch, Zahedan, 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 life-threatening 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.
Keywords
F13 deficiency
Central nervous system (CNS) bleeding
Intracranial hemorrhage
Extracranial hemorrhage
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Journal of Isfahan Medical School
Volume 31, Issue 263 - Serial Number 263
November and December 2014
Pages 1985-1993

  • Receive Date 25 July 2013
  • Accept Date 06 April 2022