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.

Coverage and perceptions of Medical Sciences students towards hepatitis B virus vaccine in Sana'a City, Yemen.

OBJECTIVE: The present study was conducted to estimate vaccination coverage against hepatitis B virus and the perceptions of 1198 medical sciences students in Sana'a City, Yemen. METHODS: Only those who practice clinical training or are in contact with body fluids were included. The students were enrolled in the Faculty of Medicine and Health Sciences, Sana'a University, Republic of Yemen. Data was collected from 1999-2000. Arabic pre-tested questionnaire forms were completed by 840 students at a response rate of 70.6%. RESULTS: The study revealed a reported vaccination rate of 29.5%. The rate among Faculty of Medicine and Health Sciences students was 32.3%, whereas only 21.3% among the students of High Institute of Health Sciences. Students of dentistry attained the highest rate of vaccination (38.8%), while nursing students of the High Institute of Health Sciences achieved the lowest rate (17.1%). Rate of vaccination (46.6%) among female students was significantly higher than male students (22.3%) with a P- value of 0.0001. Medical assistants of the High Institute of Health Sciences scored the best (56%) in terms of knowledge, medical laboratory sciences students achieved the highest (43.6%) in attitude and dentistry students had the highest scores (35.5%) in practices. The mean knowledge of females and males was comparable, however, females achieved higher attitudes and practices. Final stage students attained better attitude scores than the pre-final and intermediate students. CONCLUSION: Vaccination coverage of medical sciences students in Sana'a City, Yemen is low. Knowledge of medical assistants is the best, attitude of medical laboratory sciences students and practices of dental students is the highest. Attitudes and practices of female students are better than that of males.