Patients with coagulation FACTOR(s) deficiency who use coagulation therapy are susceptible to forming inhibitors against coagulation FACTOR(s). In this survey we detected FACTOR V and VIII inhibitor in ten patients with combined deficiency of FACTORs V and VIII from north east of Iran (Khorassan province). It was revealed in our survey that eight patients had both FACTOR V and FACTOR VIII inhibitors and two patients had none. Because FACTOR V and FACTOR VIII share approximately 40% amino acid sequence homology in their A and C domains, it remains to be elucidated if it is one molecule that recognizes both FACTOR V and VIII or whether there are two inhibitor molecules against common sites.

This review summarizes current data on the pathomechanisms and new genetic findings of combined FACTOR V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting FACTORs comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined FACTOR V and VIII deficiency (F5F8D) was reported by Oeri et al in 1954. That is distinct from the coinheritance of both FV deficiency (parahaemophilia) and FVIII deficiency (haemophilia A) that has been reported in four families. Individuals who present with this phenotype have between 5 and 30% of normal plasma levels of FV and FVIII antigen and ACTIVITY, whereas the level of other plasma proteins are not altered. Total numbers of affected individuals are less than 150 cases all over the world. At first it was assumed that deficiency of protein C inhibitor was a responsible cause, but further investigations revealed that it was due to mutations called ERGIC-53 and LMAN-1.

Background: FACTOR V G1691A (FV Leiden), FII GA20210, and methylenetetrahydrofolate reductase (MTHFR) C677T mutations are the most common genetic risk FACTORs for thromboembolism in the Western countries. However, there is rare data in Iran about cerebral venous and sinus thrombosis (CVST) patients. The aim of this study was to evaluate the frequency of common genetic thrombophilic FACTORs in CVST patients.Materials and Methods: Forty consequently CVST patients from two University Hospital in Isfahan University of Medical Sciences aged more than 15 years from January 2009 to January 2011 were recruited. In parallel, 51 healthy subjects with the same age and race from similar population selected as controls. FV Leiden, FII GA20210, MTHFR C677T, and FV Cambridge gene mutations by polymerase chain reaction technique were evaluated in case and control groups.Results: FV Leiden, FII GA20210, and FV Cambridge gene mutations had very low prevalence in both case (5%, 2%, 0%) and control (2.5%, 0%, 0%) and were not found any significant difference between groups. MTHFR C677T mutations was in 22 (55%) of patients in case group and 18 (35.5%) of control group (P=0.09).Conclusion: This study showed that the prevalence of FV Leiden, FII GA20210, and FV Cambridge were low. Laboratory investigations of these mutations as a routine test for all patients with CVST may not be cost benefit.

Dear Editor I read the fascinating letter entitled: 'All That Glitters is Not Gold: A Pregnant Woman with a Rare Inherited Bleeding Disorder" published in the latest issue Iranian Journal of Blood and Cancer with great attention. . .

Nowadays, in most earthquake resistant design codes, structures are permitted to experience significant inelastic deformations under severe earthquake. Structural experts believe that if structures are designed properly, under severe earthquake, they can deflect in inelastic limits, and, as a result, can dissipate most of the earthquake energy.A general way to evaluate the inelastic behavior of structures is using nonlinear dynamic analyses. These kinds of analysis are usually time consuming and uneconomic and need a high level of knowledge to be undertaken. Thus, it is not always possible for practicing engineers to perform these sorts of analyses. In order to solve the problem, earthquake resistant design codes permit use of a reduction FACTOR (R). This FACTOR, named the response modification FACTOR, reduces the earthquake design force so that the response of the structure is assumed elastic. In this study, the ductility reduction FACTOR of ordinary and special concentrically braced frames, using a combination of V and inverted V bracing systems, is investigated. According to the results, the maximum height of ordinary frames, which are braced using a combination of V and inverted V bracing systems, can increase up to 15 meters. This value is larger than that proposed by ASCE7 (10.7 m). Also, results indicate that using ordinary frames, which are braced by a combination of special inverted V and V braced systems, can have a saving of about 0 to 29 percent using materials for frames from 1 to 16 stories, in comparison with ordinary frames. According to the results of this study, the response modification FACTOR proposed by the Iranian seismic design code (2800 standard fourth edition), (R=5.5), is more logical than the one proposed by ASCE7 (R=6). Unfortunately, for frames braced by a combination of special inverted V and V braced systems, when the stories of the frames increase up to 10, the expected ductility demand cannot be achieved. So, as a result, for frames with more than 10 stories, the lower response modification FACTOR should be used. In addition, frames taller than 10- stories do not experience specified target displacement and collapse before reaching the preferred mechanism. This phenomenon shows the necessity of using a different response modification FACTOR for frames taller than 10 stories.

Background: Activated protein C (APC) inactivates FACTOR V (FV) by cleavage of its heavy chain at Arg306, Arg506, Arg679, and Lys994. Mutational changes, which abolish APC cleavage sites, may predispose thrombosis by altering the inactivation process of FV. FV Leiden (FVL) (Arg506Glu) has been demonstrated as a strong risk FACTOR for thrombosis. In the current study, we have studied whether mutations in the cleavage sites of FV for APC, not due to FVL, would have a role in presenting APC resistance (APCR) and initiation of a cerebral thrombotic event. Methods: A group of 22 patients with a history of cerebral venous thrombosis (CVT), who were not carriers of FVL enrolled in the study. The patients who had conditions associated with acquired APCR were excluded from the study. APCR test was performed on the remaining 16 patients, which showed APCR in 4 plasma samples. DNA sequencing was performed on four exons of FV of APCR patients, encoding Arg306, Arg506, Arg679, and Lys994. Results: Mutations were not found within nucleotides encoding the cleavage sites; neither was found within their close upstream and downstream sequences. Conclusion: Our results show that polymorphisms affecting cleavage sites of FV other than Arg506Glu it would be less likely to be the basis for APCR and its increased thrombosis susceptibility. In addition, it emphasizes on the importance of screening for APCR in the patients diagnosed with CVT.

Introduction & Objective: FACTOR V Leiden and prothrombin mutation are not common but they are involved in pediatric thrombosis. The aim of this study was to evaluate the frequency of FACTOR V Leiden & prohtrombin mutation in healthy population of Shiraz, south of Iran.Materials & Methods: In this cross-sectional study 195 healthy people (97 female and 98 male) were randomly selected. Peripheral white blood cells obtained from 5 ml blood contained 1-2 mg/ml K2- EDTA. Genomic DNA extraction was performed following the protocol described by Miller et al. PCR amplification was carried out in 25μl reaction volume containing 0.5 units Taq polymerase, 200μM dNTP, 500 μM of each of the previously described primers. After initial denaturation, 35 cycles at 95◦c for 30s, and 72◦c for 20s and followed extention by 72 for 10 min were performed. About 10μl of PDR product was digested with MNI I or Mbo restriction enzymes.Results: In this study we determined FACTOR V Leiden in 8 (4.1%) and prothrombin mutation in 6 individual (3.07%) of 198 cases in heterozygous form. No homozygous was seen for any of the mutations. Only one case presented a double heterozygous for FACTOR V and prothrombin in this cohort. Conclusion: Several studies of FACTOR V leiden and prothrombin mutations in the East of Asia showed the higher frequency of these mutations in Iran.

Background and Objective: The venom of many Viperidae snake appear to contain proteins that affect blood coagulation. The aim of this study was to identify and isolate a blood coagulation FACTOR V activator present in the venom from Vipera lebetina.Material and Methods: FACTOR V activator was purified from 200 mg of crude venom by three steps of chromatorgraphy which included: gel filtration on sephadex G100, Ion exchange chromatography on DEAE and cellulose and affinity chromatography on heparin agarose. The effect of FACTOR V activator on human FACTOR V was studied by measuring the amidiolytic ACTIVITY of the produced thrombin by activated FACTOR V (Va).Results: The results of SDS – PAGE identified an activator of FACTOR V as a single protein band with a molecular weight of 29 KDa. This compound was converted, in the presence of calcium ions, to active FACTOR Va. It also had arginine esterase ACTIVITY toward substrate BAEE (NX-benzoyl arginine ethyester) and a weak amidase ACTIVITY on S-2222 (benzoylle-glu-Gly-Arg-p- nitroanilide).Conclusion: The results of this study showed that the isolated FACTOR is a specific activator on FACTOR V.

Normal hemostasis requires balanced regulation of prothrombotic and antithrombotic FACTORs. Inherited alteration of FACTOR V and prothrombin gene, the G20210A mutation, increases the resistance of FACTOR V to degradation and booster production of prothrombin respectively. These alterations can increase hypercoagulability leading to thrombotic consequences. We aimed to assess the frequencies of these mutations in a group of the population of southern Iran. In total, 198 healthy volunteers with the age range of 1-64 years were selected and screened for FACTOR V Leiden and prothrombin mutations using polymerase chain reaction and restriction fragment length polymorphism techniques. The carrier frequencies for FACTOR V Leiden and prothrombin mutation in the studied cohort were 4.1% and 3.07%, respectively. In the studied area, the allele frequency of FACTOR V is higher than the prothrombin G20210A mutation (0.0204 v 0.0153). According to the data and Hardy-Weinberger equation, the total risk of thrombosis caused by homozygosity and heterozygosity of FACTOR V Leiden, prothrombin G20210A mutation and compound heterozygosity of these mutations are about 1 in 500 individuals.