Golestan University of Medical Sciences Repository

Recommendation prophilaxis in all congenital factor XIII deficiency with abnormal ureaclot lysis test

Mirbehbahani, N. (2014) Recommendation prophilaxis in all congenital factor XIII deficiency with abnormal ureaclot lysis test. HAEMOPHILIA, 20 (2, SI). pp. 37-38.

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Abstract

Introduction: Routine prophilaxis with FXIII concentrate is recommended in all individuals with FXIII levels of <1 IU dL⁻¹ from the time of diagnosis and in some severely affected patients with FXIII levels of 1–4 IU dL⁻¹. In Iran, ureaclot lysis test (FXIII screen) is the most available test and reliable assays are not accessible because of limitations of laboratory equipment and the aim of this study was to determine cases that need to prophilaxy with attention to limitations of laboratory equipment in Iran. Methods: There were 110 patients with hemophilia and rare bleeding disorders in Golestan pronice, Iran, involving 65 hemophila A, 13 hemophilia B and 32 rare bleeding disorders that 6 cases had FXIII deficiency. 5 patients with FXIII deficiency had on demand therapy and one of them had prophilaxy treatment due to history of brain hemorrhage. The interval of treatment and drug administration were compared between two groups. Results: The mean of interval of treatment in on demand group was 8 weeks. While interval treatment was 4 weeks for patient on prophilaxy. Then the times of treatment in prophilaxy were just twofolds of on demand for any patient. Discussion/Conclusion: According to complication and expensive treatment of brain hemorrhage, it was recommended prophilaxy treatment by fibrogammin® P (10–20 IU kg⁻¹ every 4 weeks) for all patients with abnormal ureaclot lysis test until providing laboratory equipment for FXIII assay. Disclosure of Interest: None Declared.

Item Type: Article
Subjects: موارد کلی
Divisions: معاونت تحقیقات و فناوری
Depositing User: GOUMS
Date Deposited: 27 Apr 2015 08:09
Last Modified: 23 Jun 2017 15:35
URI: http://eprints.goums.ac.ir/id/eprint/3730

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