PREVALENCE, LABORATORY DIAGNOSIS AND TREATMENT OF FACTOR XIII DEFICIENCY IN IRAN

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DORGALALEH AKBAR; ESMAEILI REIKANDE SAMIRA; TABIBIAN SHADI; VARMAGHANI BIJAN; SHAMS MAHMOOD; TAVASOLI BEHNAZ

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Journal: Razi Journal of Medical Sciences Year:2016 | Volume:23 | Issue:147 Page(s): 44-55

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Title

PREVALENCE, LABORATORY DIAGNOSIS AND TREATMENT OF FACTOR XIII DEFICIENCY IN IRAN

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Keywords

FACTOR XIII DEFICIENCYQ1

LABORATORY DIAGNOSISQ3

CLINICAL MANIFESTATIONQ3

Abstract

Background: FACTOR XIII DEFICIENCY is a bleeding disorder that its prevalence in the general population is about 1 in 2 million people around the world. In Iran, the high rate of consanguineous marriages lead to high rate of disorder with 473 factor XIII deficient patients that is about 12 times higher than the global prevalence of disorder.Methods: The study is a comprehensive review of all aspects of FACTOR XIII DEFICIENCY in Iran.Results: The distribution of the disease in different parts of Iran shows that Sistan and Baliuchistan province not only has the largest number of patients with FACTOR XIII DEFICIENCY in Iran, but also the world's highest incidence of the disease has been reported in this area. In Iranian patients, bleeding from the umbilical cord, hematoma and prolonged bleeding are the most common clinical presentations. Several disease-causing mutations are observed in Iranian patients with the most common mutation being FACTOR XIII DEFICIENCY with Trp187Arg in southeast of Iran. Traditionally, patients with FACTOR XIII DEFICIENCY were treated with fresh frozen plasma or cryoprecipitate in Iran, until 2009, when factor XIII concentrates became available for the treatment of patients. Several studies on the efficacy and safety of prophylactic regimes in different situations with valuable findings have been evaluated.Conclusion: This comprehensive study that covered all aspects of FXIII deficiency provided a suitable backbone for the diagnosis and management of FXIII deficiency in Iran.

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APA: Copy

DORGALALEH, AKBAR, ESMAEILI REIKANDE, SAMIRA, TABIBIAN, SHADI, VARMAGHANI, BIJAN, SHAMS, MAHMOOD, & TAVASOLI, BEHNAZ. (2016). PREVALENCE, LABORATORY DIAGNOSIS AND TREATMENT OF FACTOR XIII DEFICIENCY IN IRAN. RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES), 23(147), 44-55. SID. https://sid.ir/paper/11159/en

Vancouver: Copy

DORGALALEH AKBAR, ESMAEILI REIKANDE SAMIRA, TABIBIAN SHADI, VARMAGHANI BIJAN, SHAMS MAHMOOD, TAVASOLI BEHNAZ. PREVALENCE, LABORATORY DIAGNOSIS AND TREATMENT OF FACTOR XIII DEFICIENCY IN IRAN. RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES)[Internet]. 2016;23(147):44-55. Available from: https://sid.ir/paper/11159/en

IEEE: Copy

AKBAR DORGALALEH, SAMIRA ESMAEILI REIKANDE, SHADI TABIBIAN, BIJAN VARMAGHANI, MAHMOOD SHAMS, and BEHNAZ TAVASOLI, “PREVALENCE, LABORATORY DIAGNOSIS AND TREATMENT OF FACTOR XIII DEFICIENCY IN IRAN,” RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES), vol. 23, no. 147, pp. 44–55, 2016, [Online]. Available: https://sid.ir/paper/11159/en

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