FACTOR XIII DEFICIENCY IN SISTAN AND BALUCHISTAN PROVINCE

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NADERI M.; IMANI M.; ESHGHI P.; DORGALALEH A.; TABIBIAN SH.; ALIZADEH SH.; SANEI MOGHADDAM E.; MIRI MOGHADDAM E.

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Journal: The Scientific Journal of Iranian Blood Transfusion Year:2013 | Volume:10 | Issue:3 (41) Page(s): 282-288

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Title

FACTOR XIII DEFICIENCY IN SISTAN AND BALUCHISTAN PROVINCE

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Keywords

FACTOR XIII DEFICIENCYQ3

BLOOD COAGULATION DISORDERSQ2

BLEEDINGQ2

Abstract

Background and Objectives: FACTOR XIII DEFICIENCY is one of the rarest BLEEDING disorders with an estimated prevalence of 1 in 1-3 million in the general population. The main clinical manifestations of the disease are delayed wound BLEEDING, recurrent miscarriage, intracranial BLEEDING, and umbilical cord BLEEDING. The prevalence of the disease in the regions such as Sistan and Baluchistan with consanguinity marriages is higher. The aim of this study was to assess the diagnosis and treatment of FACTOR XIII DEFICIENCY in Sistan and Baluchistan province.Materials and Methods: This descriptive study was performed on 205 patients with the severe FACTOR XIII DEFICIENCY. The diagnosis of the disease was done by a wide spectrum of characteristics including family history, clinical manifestations, laboratory tests, clot solubility in 5 M urea or monochloroacetic acid 1% environments.Results: It seems that Khash city has the highest prevalence of the disease worldwide. The molecular analysis of the patients showed that an identical homozygote mutation TGG®CGG at codon 187 in exon 4 of catalytic A subunit is responsible of this deficiency. The common manifestations of the disease at time of diagnosis were umbilical BLEEDING (84.4%), deep soft tissue haematoma (54.4%), and less frequently gum BLEEDING and finally ecchymosis.Conclusions: It seems that Sistan and Baluchistan province has the highest prevalence of FACTOR XIII DEFICIENCY worldwide with a dramatic increase of 46 cases in 2008 to 205 patients in 2012 that alarmed the absence of a screening test in this region.

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APA: Copy

NADERI, M., IMANI, M., ESHGHI, P., DORGALALEH, A., TABIBIAN, SH., ALIZADEH, SH., SANEI MOGHADDAM, E., & MIRI MOGHADDAM, E.. (2013). FACTOR XIII DEFICIENCY IN SISTAN AND BALUCHISTAN PROVINCE. THE SCIENTIFIC JOURNAL OF IRANIAN BLOOD TRANSFUSION ORGANIZATION (KHOON), 10(3 (41)), 282-288. SID. https://sid.ir/paper/78646/en

Vancouver: Copy

NADERI M., IMANI M., ESHGHI P., DORGALALEH A., TABIBIAN SH., ALIZADEH SH., SANEI MOGHADDAM E., MIRI MOGHADDAM E.. FACTOR XIII DEFICIENCY IN SISTAN AND BALUCHISTAN PROVINCE. THE SCIENTIFIC JOURNAL OF IRANIAN BLOOD TRANSFUSION ORGANIZATION (KHOON)[Internet]. 2013;10(3 (41)):282-288. Available from: https://sid.ir/paper/78646/en

IEEE: Copy

M. NADERI, M. IMANI, P. ESHGHI, A. DORGALALEH, SH. TABIBIAN, SH. ALIZADEH, E. SANEI MOGHADDAM, and E. MIRI MOGHADDAM, “FACTOR XIII DEFICIENCY IN SISTAN AND BALUCHISTAN PROVINCE,” THE SCIENTIFIC JOURNAL OF IRANIAN BLOOD TRANSFUSION ORGANIZATION (KHOON), vol. 10, no. 3 (41), pp. 282–288, 2013, [Online]. Available: https://sid.ir/paper/78646/en

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