Socialization During the COVID-19 Pandemic: The Role of Social and Scientific Networks During Social Distancing.

In the COVID-19 era, while we are encouraged to be physically far away from each other, social and scientific networking is needed more than ever. The dire consequences of social distancing can be diminished by social networking. Social media, a quintessential component of social networking, facilitates the dissemination of reliable information and fighting against misinformation by health authorities. Distance learning, telemedicine, and telehealth are among the most prominent applications of networking during this pandemic. Additionally, the COVID-19 pandemic highlights the importance of collaborative scientific efforts. In this chapter, we summarize the advantages of harnessing both social and scientific networking in minimizing the harms of this pandemic. We also discuss the extra collaborative measures we can take in our fight against COVID-19, particularly in the scientific field.

Inherited Platelet Function Disorders (IPFDs).

BACKGROUND: Inherited platelet function disorders (IPFDs) are a wide spectrum of qualitative platelet disorders with variable bleeding tendency, ranging from mild bleeding to severe life-threatening episodes. Diagnosis and classification of IPFDs is a challenge worldwide. The present study aims to present a proper classification, describe the molecular basis and clinical presentations as well as some diagnostic clues for these disorders. METHODS: All relevant publications were searched using appropriate keywords. RESULTS: IPFDs can be divided into four major groups including defects of platelet surface glycoproteins, platelet granules and secretion disorders, platelet signaling defects, and transcription-related platelet disorders. Some of these disorders, such as Glanzman thrombasthenia, are more common, with severe bleeding, while most of these disorders are extremely rare with mild bleeding. CONCLUSIONS: A proper classification, accompanied by familiarity with diagnostic clinical and laboratory features of IPFDs, can be helpful in in-time and exact diagnosis of these complicated bleeding disorders.

Effects of a peer education on cardiac self-efficacy and readmissions in patients undergoing coronary artery bypass graft surgery: a randomized-controlled trial.

AIM: This study aimed to investigate the effects of a peer education on cardiac self-efficacy (CSE) and readmission of the patients undergoing bypass surgery. BACKGROUND: Self-efficacy is an antecedent vital factor in both initiating and maintaining healthy behaviours. It significantly improves after effects of heart attacks, anxiety and diminishes the possibility of readmission. DESIGN: This study is a randomized-controlled trial. METHOD: Sixty patients undergoing bypass surgery were chosen and assigned equally into the control and intervention groups. While routine education was presented to the patients in the control group, intervention group were taught using the peer education in two sessions. CSE of all the selected patients was assessed orderly in 5 days, 4 weeks and 8 months after surgery. Moreover, their readmission was investigated after 8 months from surgery. Data was collected using demographic and the CSE scale. Data were also analysed by using χ2 , Kolmogorov-Smirnov and repeated measures analysis of variance tests. RESULTS: The mean score of CSE in the intervention group was significantly different from the corresponding number in the control group in all three stages of data collecting (p < 0·001). Compared to the control group, a smaller number of the patients in the intervention group were readmitted to the hospitals after 8 months (p = 0·011). CONCLUSIONS: Implementation of peer education has positive effects on CSE in patients who have bypass surgery and reduces their hospital readmission. It can be beneficial to apply this method as an educative-supportive approach in cardiac surgery fields. RELEVANCE TO CLINICAL PRACTICE: Implementation of peer education has positive effects on CSE in these patients and reduces their hospital readmission.

Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency.

With 473 patients, Iran has about one third of the world's patients with severe congenital factor XIII (FXIII) deficiency. A considerable number of patients with FXIII deficiency (FXIIID) are affected by life-threatening bleeding episodes, such as central nervous system (CNS) bleeding or recurrent miscarriage and umbilical cord bleeding (UCB), that cause a high rate of morbidity and mortality in Iranian patients with FXIIID. Among 317 Iranian patients with FXIIID, 145 cases experienced 166 CNS bleeds (CNSBs) that recurred in 21 cases. CNSB caused different types of neurological complications in 69 patients. A total of 62 miscarriages were observed in 24 women of childbearing age, and 21 deaths were observed due to umbilical cord bleeding or mucosal bleeding. In fact, 49 deaths (15.4 %) were observed in these patients, which highlight the importance of early diagnosis and intensive health care among patients with FXIIID.

Laboratory Diagnosis of Factor XIII Deficiency, Routine Coagulation Tests with Quantitative and Qualitative Methods.

BACKGROUND: Factor XIII (FXIII) deficiency is a severe bleeding disorder with normal routine coagulation tests that makes diagnosis of the disorder complicated. After normal results in routine coagulation tests, clot solubility test, and FXIII activity, antigen assays along with molecular methods can be used for precise diagnosis of disorder. In the present study, we described routine coagulation tests along with clot solubility test and FXIII activity and antigen assays. METHODS: Data were collected from all relevant publications until 2015. RESULTS: All routine coagulation tests including prothrombin time (PT), partial thromboplastin time (PTT), thrombin time (TT), and platelet count are normal in FXIII deficiency (FXIIID) but different conditions such as blood collection, transport, and storage can result in abnormal results in these first line screening tests. In addition to these tests, clot solubility tests as the most common screening tests of FXIIID can influenced by different factors including clotting and solubilizing agents. Different commercial kits are available for FXIII activity and antigen assays with different sensitivity and specificity which could affect diagnosis of FXIIID. CONCLUSIONS: Results of routine coagulation tests as well as clot solubility tests along with specific coagulation tests can affect diagnosis of FXIIID; therefore, all steps of these tests should be under control.

Genetic risk factors in patients with deep venous thrombosis, a retrospective case control study on Iranian population.

BACKGROUND: Venous thromboembolism (VTE) could be manifested as deep venous thrombosis (DVT) or pulmonary embolism (PE). DVT is usually the more common manifestation and is usually formation of a thrombus in the deep veins of lower extremities. DVT could occur without known underlying cause (idiopathic thrombosis) which could be a consequence of an inherited underlying risk factor or could be a consequence of provoking events, such as trauma, surgery or acute illness (provoked thrombosis). Our aim in this study was to assess the impact of some previously reported genetic risk factors including, methylenetetrahydrofolate reductase (MTHFR) C677T and A1298C, plasminogen activator inhibitor-1(PAI-1) 4G/5G, prothrombin 20210 and FV Leiden on occurrence of DVT in a population of Iranian patients. METHODS: This long-term study was conducted on 182 patients with DVT and also 250 age and sex matched healthy subjects as control group. The diagnosis of DVT was based on patient's history, clinical findings, D-dimer test, and confirmed by Doppler ultrasonography. After confirmation of DVT, both groups were assessed for the five mentioned mutations. The relationship between mutations and predisposition to DVT was calculated by using logistic regression and expressed as an OR with a 95 % confidence interval (CI). RESULTS: Our results revealed that FV Leiden (OR 6.7; 95 % CI = 2.2 to 20.3; P = 0.001), MTHFR C677T (OR 6.0; 95 % CI = 2.2 to 16.4; P < 0.001), MTHFR A1298C (OR 8.3; 95 % CI = 4.4 to 15.8; P < 0.001), and PAI-1 4G/5G (OR 3.8; 95 % CI = 2.1 to 7.2; P < 0.001) mutations were all significantly associated with an increased risk of DVT. Prothrombin 20210 was found in none of the patients and controls. CONCLUSION: Our findings suggest that genetic risk factors have a contributory role on occurrence of DVT.

Congenital factor V deficiency: comparison of the severity of clinical presentations among patients with rare bleeding disorders.

BACKGROUND: Factor V deficiency (FVD) is a rare bleeding disorder (RBD) mostly present in regions with a high rate of consanguinity. FVD after FXIII deficiency is the next more prevalent RBD in Sistan and Baluchistan (S&B) in southeastern Iran. The aim of this study was to evaluate the clinical manifestations and severity of bleeding diathesis in patients with FVD. METHODS: This descriptive study was conducted on 23 patients with FVD in S&B province. FVD was diagnosed by clinical findings and routine laboratory tests. Bleeding diatheses were classified into three grades (I-III) depending on the severity of symptoms. The severity of bleeding episodes in our patients was compared with other RBDs. RESULT: Based on residual plasma FV activity, 6 (26%), 16 (69.5%) and 1 (4.5%) patients had mild, moderate and severe factor deficiency, respectively. 24% of the patients had grade III life-threatening bleeding episodes which in comparison with FVII deficiency (17.4%) and FI deficiency (21%) had a higher incidence, and in comparison with FX deficiency (41.7%) and FXIII deficiency (63.1) had a lower incidence. Grade II and grade I bleeding diathesis were observed in 56.2 and 16.7% of the patients, respectively. CONCLUSION: FVD is the second most common type of RBD in S&B province and grade II bleeding episodes were the major bleeding presentation and observed in more than half of the patients.

Application of Haddon strategy training on self-care behavior and disease consequences in heart failure.

OBJECTIVES: Heart failure affects the physical, physiological, social, and activities of individuals. The study aimed to teach preventive behavior with Haddon strategy on self-care behavior and the consequences of heart failure. METHODS: In this clinical trial, 96 patients with heart failure were randomly assigned to two groups. The Haddon group was educated with Haddon prevention strategies three times a week, for 60 minutes. A demographic questionnaire, self-care index, and consequences checklist were filled in both groups at the start of the study, discharge time, and one month after the last training session. Statistical analysis was done using independent t-test, paired t-test, chi-square, Fisher and analysis of covariance, Friedman, and Cochran Q in SPSS with version 23. RESULTS: Self-care behavior in the Haddon group after training significantly increased (p < 0.001). Disease consequences were significantly reduced in the Haddon group after training (p < 0.05). The consequences of the disease in Haddon group decreased during 4 weeks of monitoring and the changes were significant (p < 0.001). CONCLUSION: It is suggested that this educational method could be used to increase self-care behavior, prevent hospitalizations, decrease symptoms, and improve quality of life for patients with heart failure. PRACTICE IMPLICATIONS: Nurses can identify and manage risk factors for heart disease using the Haddon strategy.

Incidence and Risk Factors of Ventilator-Associated Pneumonia among Patients with Delirium in the Intensive Care Unit: A Prospective Observational Study.

INTRODUCTION: The incidence and risk factors for ventilator-related pneumonia (VAP) in patients with delirium are deficient, and there is a lack of in-depth knowledge of the impact of VAP on outcomes in this population. We investigated the incidence, risk factors, and outcomes of VAP in patients with delirium. MATERIALS AND METHODS: This prospective observational study was performed in a surgical ICU at Be'sat Hospital in Hamadan, Iran, between 2018 and 2019. A total of 108 patients with delirium were identified using the Confusion Assessment Method (CAM) for the ICU and Intensive Care Delirium Screening Checklist (ICDSC) and enrolled in this study. The association between VAP and delirium, risk factors, and outcomes (ICU length of stay and ICU mortality) for VAP were investigated using the Cox proportional hazards model and logistic and simple linear regression analyses with a 95% confidence interval. RESULTS: Of 108 delirium patients, 86 patients (79.6%) underwent mechanical ventilation (MV) and 16 patients (18.6%) experienced VAP during ICU stay. The median onset of VAP was 6.5 (IQR 4.2-7.7) days after intubation. Delirium patients with VAP stayed longer in the ICU (21.68 ± 4.26 vs.12.93 ± 1.71, P < 0.001) and also had higher ICU mortality (31.25% vs. 0%, P < 0.001) than subjects without VAP. According to multivariate cox regression, the expected HR for VAP was 53.5% lower for patients with early-onset delirium than in patients with late-onset delirium (HR: 0.465, 95% CI: 0.241-0.894, P=0.022). However, the expected hazard for VAP was 1.854 times and 4.604 times higher in patients with longer ICU stay (HR: 1.854, 95% CI: 1.689-3.059, P=0.032) and in patients with a prolonged MV duration (HR: 4.604, 95%CI: 1.567-6.708, P=0.023). CONCLUSION: According to the results, there seems to be an inverse relationship between early onset of delirium and VAP. This finding cannot be conclusively cited, and more studies in this filed should be conducted with a larger sample size. Furthermore, VAP in delirium patients is associated with increases in poor outcomes (higher ICU mortality) and the use of medical resources (longer stay in the ICU and MV duration).

Factors Associated With Hope and Quality of Life in Patients With Coronary Artery Disease.

BACKGROUND: Psychological resources such as hope have been suggested to affect quality of life (QoL) positively in patients with heart disease. However, little information regarding the relationship between these two constructs is available. PURPOSE: This work was aimed at examining the factors associated with hope and QoL in patients with coronary artery disease. METHODS: In this descriptive work, perceived QoL and hope were assessed in 500 patients with heart disease. The information was collected using the McGill QoL Questionnaire, demographic variables, and the Herth Hope Index. The Pearson correlation test and general linear model were used to examine correlations through SPSS Version 22. RESULTS: A considerable correlation was discovered between QoL and hope (r = .337, p < .001). Multivariate analyses with regression revealed that religious beliefs and social support both had significant and positive effects on the total perceived hope of patients and that patient age had a considerable negative impact on QoL (p < .05). None of these factors had a significant impact on hope (p < .05). In addition, the total QoL had a significant and positive effect on patient feelings and thoughts, whereas the physical problems component of QoL had a significant and negative effect on hope (p < .05). Participants with higher levels of education reported more hope. CONCLUSIONS: QoL relates significantly to self-perceived hope in patients. Understanding QoL and hopefulness in patients with coronary artery disease has implications for nurses and other healthcare professionals.

The Antimicrobial and Healing Effect of Scrophularia striata Boiss Hydroalcoholic Extract on First- and Second-Grade Pressure Wounds in Patients with Brain and Spinal Cord Injury: A Randomized Clinical Trial.

Materials and Methods: This double-blind clinical trial study was performed on grade 1 and 2 pressure wounds in 120 patients with cerebral-spinal cord lesions. The patients were randomly divided into four groups (n = 30). Topical treatments in all groups were performed twice a day. These groups included experiment 1 (SHE + phenytoin), experiment 2 (SHE + SHE), control (phenytoin + phenytoin), and placebo (eucerin + phenytoin). After evaluating the effect of SHE on wound healing, its antibacterial activity was determined by the standard agar well diffusion method. Results: Patients in each group in this study did not significantly differ in demographic and clinical variables. Complete wound healing by the 10th day of the intervention occurred in 63%, 100%, and 27% of patients in experimental 1, experimental 2, and control groups, respectively. In contrast, the placebo group had no complete wound healing until the 10th day. Topical application of SHE, twice a day in the experimental 2 groups, had a higher potency to heal wounds and reduce the duration of complete wound healing in patients compared with other groups. Conclusion: SHE, as a novel treatment option, has good potential to accelerate the healing of first- and second-degree pressure wounds in patients with brain-spinal cord injuries.

The effects of foot massage on hemodialysis patients' sleep quality and restless leg syndrome: a comparison of lavender and sweet orange essential oil topical application.

OBJECTIVES: Hemodialysis (HD) patients suffer more sleep problems (poor sleep quality and restless leg syndrome [RLS]). Complementary therapy, especially massage with aromatherapy oil is one of the non-pharmacological treatment options with less adverse effects than routine methods. The purpose of this study was to determine the effects of foot massage with of lavender and orange essential oil on HD patients' sleep quality and RLS. METHODS: This is a double blind randomized controlled trial on 105 HD patients was conducted at a large educational hospital in Iran, Hamadan province between January and September 2017. Patients divided into three groups with random allocation (35 participants per groups in lavender, orange, and control group). Foot massage during HD with lavender and orange essential oil was administered to the patients three times a week for three weeks, and every massage lasted half an hour. The control group received routine care. Before the intervention, the end of the first, second, and third weeks Pittsburgh Sleep Quality Index (PSQI) and RLS questionnaire were completed for all three groups. Data were statistically analyzed with Independent Samples t-test, chi-square test and repeated-measures analysis of variance (ANOVA) by SPSS version 16. RESULTS: The mean score of sleep quality and RLS in the intervention groups were significantly different compare with the control group in all three time of data collecting (p<0 001). CONCLUSIONS: Aromatherapy prepared with lavender oil and sweet orange may be recommended to increase sleep quality and RLS level of the HD patients.

The effect of video-based educational program on satisfaction and comfort in patients undergoing transradial coronary angiography: A single-blinded, randomized controlled trial.

Cardiac catheterization has been the most widely used diagnostic method, and in most cases, invasive diagnostic methods are associated with stress and concern that can affect the satisfaction and comfort of patients undergoing transradial coronary angiography. In this randomized controlled trial, a total of 89 patients undergoing transradial coronary angiography were recruited using convenience sampling. Then, the patients were assigned to two groups of intervention and control using blocked randomization, as those in the intervention group received video-based patient education and those in the control group received no training. Data were collected using the Persian version of the patient satisfaction questionnaire and the visual analog scale. The variables were measured before, 2, 4, and 8 hours after the angiography. The results indicated that, after the intervention, the mean scores of satisfaction and comfort in the intervention group were higher than in the control group (P < .001). Video-based educational strategies should be integrated into the nursing interventions to improve patients' health status.

Effect of a Multistage Educational Skill-Based Program on Nurse's Stress and Anxiety in the Intensive Care Setting: A Randomized Controlled Trial.

BACKGROUND: Psychological problems such as stress and anxiety are prevalent among working nurses in the intensive care units (ICUs). This study was aimed at investigating the effects of three skill-based educational programs on stress and anxiety among critical care nurses. METHODS: Using a randomized controlled trial, 160 nurses were assigned to four groups including one control and three intervention groups. A standard skill-based educational program was delivered to three intervention groups using booklet, booklet+oral presentation, and booklet+oral presentation+clinical teaching over a period of one month to reduce different types of stress and anxiety. The control group received routine education only. Perceived stress, state anxiety, trait anxiety, and work-related stress were assessed at baseline and three times after the intervention (15 days, 3 months, and 21 months). Repeated-measure analysis of variance was used for data analysis. RESULTS: There was no significant change in the control group in terms of study variables during follow-up assessments, whereas measures of stress and anxiety were reduced after intervention in the trial groups except trait anxiety. Nurses in the mixed-method group (booklet+oral presentation+clinical teaching) showed less stress and anxiety during follow-ups. Although the stress and anxiety scores decreased in the first and second follow-ups, there was no significant reduction in the third follow-up. CONCLUSIONS: To improve the mental health and performance of the intensive care unit nurses, knowledge-based and skill-based training programs seem useful. Continuous training may help to maintain the effectiveness of these programs over time.

Effect of the Specific Training Course for Competency in Doing Arterial Blood Gas Sampling in the Intensive Care Unit: Developing a Standardized Learning Curve according to the Procedure's Time and Socioprofessional Predictors.

BACKGROUND: Standardization of clinical practices is an essential part of continuing education of newly registered nurses in the intensive care unit (ICU). The development of educational standards based on evidence can help improve the quality of educational programs and ultimately clinical skills and practices. OBJECTIVES: The objectives of the study were to develop a standardized learning curve of arterial blood gas (ABG) sampling competency, to design a checklist for the assessment of competency, to assess the relative importance of predictors and learning patterns of competency, and to determine how many times it is essential to reach a specific level of ABG sampling competency according to the learning curve. DESIGN: A quasi-experimental, nonrandomized, single-group trial with time series design. Participants. All newly registered nurses in the ICU of a teaching hospital of Tehran University of Medical Sciences were selected from July 2016 to April 2018. Altogether, 65 nurses participated in the study; however, at the end, only nine nurses had dropped out due to shift displacement. METHODS: At first, the primary checklist was prepared to assess the nurses' ABG sampling practices and it was finalized after three sessions of the expert panel. The checklist had three domains, including presampling, during sampling, and postsampling of ABG competency. Then, 56 nurses practiced ABG sampling step by step under the supervision of three observers who controlled the processes and they filled the checklists. The endpoint was considered reaching a 95 score on the learning curve. The Poisson regression model was used in order to verify the effective factors of ABG sampling competency. The importance of variables in the prediction of practice scores had been calculated in a linear regression of R software by using the relaimpo package. RESULTS: According to the results, in order to reach a skill level of 55, 65, 75, 85, and 95, nurses, respectively, would need average ABG practice times of 6, 6, 7, 7, and 7. In the linear regression model, demographic variables predict 47.65 percent of changes related to scores in practices but the extent of prediction of these variables totally decreased till 7 practice times, and in each practice, nurses who had the higher primary skill levels gained 1 to 2 skill scores more than those with low primary skills. CONCLUSIONS: Utilization of the learning curve could be helpful in the standardization of clinical practices in nursing training and optimization of the frequency of skills training, thus improving the training quality in this field. This trial is registered with NCT02830971.

Comparison the effects of inhalation and massage aromatherapy with lavender and sweet orange on fatigue in hemodialysis patients: a randomized clinical trial.

OBJECTIVES: Fatigue, regardless of the causes, could leave a negative impact on individual performance. The aim of this study was to compare the effects of inhalation and massage aromatherapy with lavender and sweet orange on fatigue among hemodialysis patients. METHODS: This study was a randomized controlled trial. The patients (96 patients) were allocated to three groups (control, an inhalation aromatherapy, and an aromatherapy massage group). A demographic questionnaire as well as the rhoten fatigue scale (RFS) was used to collect the data. The data were analyzed by descriptive and analytical statistics using SPSS version 18. RESULTS: Before the intervention, there was no significant difference among the groups regarding fatigue level (p=0.54). However, eight and 16 weeks after the study, the levels of fatigue in both experimental groups were significantly lower than the control group (p < 0.05). CONCLUSIONS: Interventions with essential oils are effective in reducing fatigue in hemodialysis patients, although the effects of aromatherapy massage on fatigue are stronger than the effects of inhalation aromatherapy.

Lived experiences of nursing students about ethical concerns regarding mobile learning in educational and clinical contexts.

The field of education has experienced a profound change following the introduction of mobile technology over the last decades, and nursing education is not an exception. This study explored the experiences of nursing students about the ethical concerns regarding the use of mobile devices for learning purposes, that is, mobile learning, in educational and clinical contexts. A qualitative phenomenological study was carried out on nursing students (n = 19) in Saveh University of Medical Sciences of Iran between December 2017 and April 2018. Data were collected through semi-structured interviews with open-ended questions. Data analysis was done using Colaizzi's 7-step method, revealing four themes and nine sub-themes including: 1) preserving professional dignity (in front of the patient, and the teacher, and preserving academic virtual identity); 2) securing informed consent and respecting personal (the patient's and teachers) autonomy; 3) proper and efficient use (observing the regulations and codes, and making educational use); and 4) avoiding harm (responsible use of class and patient data). It was revealed that using mobile technology in education could raise ethical concerns for nursing students, and this should be emphasized in nursing educational programs.

Molecular Basis of Congenital Factor XIII Deficiency in Iran.

Factor XIII deficiency (FXIIID) is an extremely rare autosomal recessive disorder that has the highest incidence in Iran. The FXIIID is primarily due to mutations in the FXIII-A gene, most of which are unique. In the current study, we report all identified mutations among Iranian patients. Among 483 patients, 366 (75.8%) were molecularly analyzed; 11 different mutations were observed. Of 11, 8 (72.7%) are missense, whereas the remaining 3 (27.3%) are deletion/insertion. Among these patients, 347 (94.9%) had the unique mutation of c.562T>C and 5 (1.4%) had the c.233G>A mutation. c.1226G>A, c.2111G>A, and c.1142T>A are also common, whereas other mutations, including 3 missense and 3 deletion/insertion, were observed only in single patient. Although, in most cases, FXIII mutations are unique and restricted to a specific family, this differs in Iran where a considerable number of identified mutations, recurrently observed, appear to be due to the high rate of consanguinity.

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.