Journal of Isfahan Medical School

Journal of Isfahan Medical School

Relationship between the Matrix Metalloproteinases and the Occurrence of Central Nervous System Bleeding in Factor XIII Deficiency

Document Type : Original Article (s)

Authors
Majid Naderi 1
Mohammad Reza Younesi 2
Akbar Dorgalaleh 3
Shaban Alizadeh 4
Ahmad Kazemi 4
Shadi Tabibian 2
Zahra Kashani-Khatib 5
1 Assistant Professor, Department of Pediatrics Hematology and Oncology, Ali Ebn-e Abi Taleb Hospital, Zahedan University of Medical Sciences, Zahedan, Iran
2 Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
3 Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences AND PhD Student, Department of Hematology, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran
4 Assistant Professor, Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
5 Department of Hematology, School of Medicine, Tarbiat Modares University, Tehran, Iran
Abstract
Background: Matrix metalloproteinase (MMP) has a crucial role in degradation of basal membrane and tissue remodeling and has a possible role in occurrence of central nervous system (CNS) bleeding. Factor XIII deficiency is an extremely rare bleeding disorder with estimated incidence of 1 per 2 million. Since central nervous system bleeding is the main cause of death among these patients, this study aimed to assess to role of MMP-9 and MMP-2 in occurrence of this bleeding in factor XIII deficiency.Methods: In this case-control study, gene expression of MMP-9 and MMP-2 was determined via quantitative real-time reverse transcription polymerase chain reaction (Q-RT-PCR) assays in 42 patients with factor XIII deficiency that were divided in two groups of with (case) and without central nervous system bleeding (control). Gene expression was compared with comparison method (2–ΔΔcCt) and glyceraldehyde 3-phosphate dehydrogenase (GAPDH) was used for standardization of gene expression.Findings: Cord bleeding was the most common bleeding episode among all the patients. Overexpression of MMP-9 was observed among 13 patients in case (72.2%) and 3 patients in control group (12.5%) that showed a statistically significant difference (P = 0.001, CI95%: 2.8-95.3).Conclusion: Patients with factor XIII deficiency have a wide spectrum of clinical presentations that has a crucial role in screening and diagnosis of the disease. According to results of this study, overexpression of MMP-9, due to polymorphism or inflammation, had a role in pathogenesis of central nervous system bleeding. Inhibition of MMP-9 may have a role in decreasing the rate of morbidity and mortality among patients with factor XIII deficiency.
Keywords
Factor XIII deficiency
Central nervous system bleeding
Matrix metalloproteinase
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Journal of Isfahan Medical School
Volume 32, Issue 311 - Serial Number 311
September and October 2015
Pages 2025-2034

  • Receive Date 13 June 2014
  • Accept Date 06 April 2022