Long-term prophylaxis in patients with severe congenital factor XIII deficiency is not complicated by inhibitor formation.

: Congenital factor XIII (FXIII) deficiency is a rare bleeding disorder accompanied by a variety of bleeding events. Severely deficient patients require regular replacement therapy. With development of FXIII concentrate, the risk of viral infections transmitted by fresh frozen plasma and cryoprecipitate is diminished, but the possibility of inhibitor development remains a challenging issue in the management of these patients. The aim of this study was to assess FXIII inhibitor development in Iranian patients with FXIII deficiency (FXIIID). This study enrolled 50 (30 women and 20 men) patients with severe congenital FXIIID from southeast Iran who underwent long-term (more than 4 years or more than 50 injections) prophylaxis with FXIII concentrate (Fibrogammin P, Dade Behring, Marburg, Germany). We evaluated plasma FXIII activity and FXIII inhibitor on day 28 after the last prophylaxis administration. The method for investigation of FXIII inhibitor was based on Bethesda assay. The mean age of the study population was 13.8 ±â€Š8.3 years. The minimum and maximum FXIII activity levels were less than 1-4.5% (mean ±â€ŠSD, 2.6 ±â€Š0.7%). Our investigations showed that all patients with severe form of FXIIID were treated without inciting inhibitor development. Despite long-term prophylaxis in the studied patients, none was found to have developed FXIII inhibitors.

Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran.

BACKGROUND: Rare bleeding disorders (RBDs) are heterogeneous disorders, mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate of consanguinity that has a high rate of RBDs. OBJECTIVE: In this study, we present prevalence and clinical presentation as well as management and genetic defects of Iranian patients with RBDs. METHODS: For this study, all relevant publications were searched in Medlin until 2015. RESULTS AND DISCUSSION: Iran has the highest global incidence of factor XIII deficiency. Factor VII deficiency also is common in Iran, while factor II deficiency, with a prevalence of 1 per ∼3 million, is the rarest form of RBDs. Factor activity is available for all RBDs except for factor XIII deficiency, in which clot solubility remains as a diagnostic test. Molecular analysis of Iranian patients with RBDs revealed a few recurrent, common mutations only in patients with factor XIII deficiency, and considerable novel mutations in other RBDs. Clinical manifestations of these patients are variable and patients with factor XIII, factor X and factor VII more commonly presented severe life-threatening bleeding, while patients with combined factor V and factor VIII presented a milder phenotype. Plasma-derived products are the most common therapeutic choice in Iran, used prophylactically or on-demand for the management of these patients. CONCLUSION: Since Iran has a high rate of RBDs with life-threatening bleeding, molecular studies can be used for carrier detection and, therefore, prevention of the further expansion of these disorders and their fatal consequence.

Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P®.

Factor XIII deficiency (FXIIID) is an extremely rare hemorrhagic disorder with a prevalence of 1/3-5 million. Management of disease is performed by fresh frozen plasma (FFP), Cryoprecipitate (CP) or FXIII concentrate (Fibrogammin P®). Our objective was to assess safety and effectiveness of Fibrogammin P® in patients with FXIIID. For this purpose we designed this long-term follow up study on a large group of patients with FXIIID. This prospective study was conducted on 213 patients with FXIIID since 2009 to 2013. Administrated dose for Fibrogammin P® according to clinical situations of patients ranged from 10 to 26 IU/kg every 4 - 6 weeks. All patients in 6-month intervals were checked for human immunodeficiency virus (HIV), hepatitis A, B and C viruses (HAV, HBV, HCV). Twelve percent of participants had at least one ICH episode until 2008 but after administration of Fibrogammin P® did not have any major bleeding or episode of ICH, except in one patient. We also had 7 females with recurrent miscarriage that were managed successfully with a dose of 10 to 26 IU/kg every 4 - 6 weeks. This dose also was quite successful in management of major and minor surgery. None of the participants showed allergic reaction during treatment. A total of 7155450 IU of Fibrogammin P® were infused but nobody was positive for HIV, HAV, HBV, and HCV. We found that Fibrogammin P® is a safe and effective therapeutic choice in management of FXIIID.

Psychometric properties of Persian version of the multifactor leadership questionnaire (MLQ).

BACKGROUND: Outstanding leadership is one of the important and vital concepts in management and educational leadership debates especially in educational organizations such as universities. Thus, effective educational leadership and adopting an appropriate tool to assess leadership in education are crucial in these institutions. The present study was conducted to develop an instrument for measuring the leadership style in faculty members. METHODS: In this descriptive cross-sectional study, content and face validity of Multifactor Leadership Questionnaire (MLQ) was examined using the opinions of 10 experienced faculty members as a panel of experts. For construct validity, 210 questionnaires were administered to faculty members of Tehran University of Medical Sciences. Confirmatory factor analysis was run using principal component analysis and Varimax rotation method. The reliability of the scale was measured through internal consistency using Cronbach's alpha formula. Confirmatory factor analysis was used to examine the construct validity of the questionnaire. Data were analyzed using SPSS v.16 and LISREL software. RESULTS: Factor analysis and expert opinion resulted in a questionnaire with 18 items across six subscales including idealized influence, inspirational motivation, intellectual stimulation, individualized consideration, contingent reward, and management by exception. The reliability coefficient of the questionnaire was acceptable (0.90). Confirmatory factor analysis showed that the scale had appropriate goodness of fit. CONCLUSION: The 18-item Educational Leadership Questionnaire was found to have acceptable validity and reliability for measuring leadership style in the faculty. It is recommended that the questionnaire be administered to a larger sample.