Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1
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 P...
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Isfahan University of Medical Sciences
2014-01-01
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| Series: | مجله دانشکده پزشکی اصفهان |
| Subjects: | |
| Online Access: | http://jims.mui.ac.ir/index.php/jims/article/view/3065 |
| _version_ | 1797722147979788288 |
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| author | Majid Naderi Akbar Dorgalaleh Shaban Alizadeh Ahmad Kazemi Shadi Tabibian Abbas Ghutaslou Meysam Kashiri Hosnieh Hosseini Maryam-Sadat Hosseini |
| author_facet | Majid Naderi Akbar Dorgalaleh Shaban Alizadeh Ahmad Kazemi Shadi Tabibian Abbas Ghutaslou Meysam Kashiri Hosnieh Hosseini Maryam-Sadat Hosseini |
| author_sort | Majid Naderi |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-12T09:44:20Z |
| format | Article |
| id | doaj.art-9d39f57b524e484caf2fdf25d7383bae |
| institution | Directory Open Access Journal |
| issn | 1027-7595 1735-854X |
| language | fas |
| last_indexed | 2024-03-12T09:44:20Z |
| publishDate | 2014-01-01 |
| publisher | Isfahan University of Medical Sciences |
| record_format | Article |
| series | مجله دانشکده پزشکی اصفهان |
| spelling | doaj.art-9d39f57b524e484caf2fdf25d7383bae2023-09-02T13:01:44ZfasIsfahan University of Medical Sciencesمجله دانشکده پزشکی اصفهان1027-75951735-854X2014-01-0131263198519931475Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1Majid Naderi0Akbar Dorgalaleh1Shaban Alizadeh2Ahmad Kazemi3Shadi Tabibian4Abbas Ghutaslou5Meysam Kashiri6Hosnieh Hosseini7Maryam-Sadat Hosseini8Associate Professor, Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, IranDepartment of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, IranAssistant Professor, Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, IranProfessor, Department of Hematology, Allied Medical School, Iran University of Medical Sciences, Tehran, IranAssociate Professor, Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, IranDepartment of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, IranDepartment of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, IranDepartment of Laboratory Sciences, School of Medical Sciences, Islamic Azad University, Zahedan Branch, Zahedan, IranDepartment of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, IranBackground: 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.http://jims.mui.ac.ir/index.php/jims/article/view/3065F13 deficiencyCentral nervous system (CNS) bleedingIntracranial hemorrhageExtracranial hemorrhage |
| spellingShingle | Majid Naderi Akbar Dorgalaleh Shaban Alizadeh Ahmad Kazemi Shadi Tabibian Abbas Ghutaslou Meysam Kashiri Hosnieh Hosseini Maryam-Sadat Hosseini Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 مجله دانشکده پزشکی اصفهان F13 deficiency Central nervous system (CNS) bleeding Intracranial hemorrhage Extracranial hemorrhage |
| title | Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 |
| title_full | Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 |
| title_fullStr | Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 |
| title_full_unstemmed | Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 |
| title_short | Central Nervous System Bleeding in Factor XIII Deficiency, Assessment the Role of Plasminogen Activator Inhibitor-1 |
| title_sort | central nervous system bleeding in factor xiii deficiency assessment the role of plasminogen activator inhibitor 1 |
| topic | F13 deficiency Central nervous system (CNS) bleeding Intracranial hemorrhage Extracranial hemorrhage |
| url | http://jims.mui.ac.ir/index.php/jims/article/view/3065 |
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