Journal of Pediatrics Review، جلد ۲، شماره ۲، صفحات ۳۱-۴۶
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عنوان انگلیسی Rare bleeding disorders: a narrative review of epidemiology, molecular and clinical presentations, diagnosis and treatment
چکیده انگلیسی مقاله Rare bleeding disorders (RBDs) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. The disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. Patients affected with RBDs are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diathesis. These disorders are usually present in regions with high rate of parental consanguinity. Despite the rare incidence of RBDs, it is necessary for physicians to be aware of these disorders. Here we aim to have a comprehensive review on general features and also the recent advances in understanding of RBDs. MEDLINE and Web of Science databases searched for English sources from 1990 to 2014, using the following keywords: rare bleeding disorder, rare inherited disorder, factor deficiency, structure, function, epidemiology, manifestations, laboratory analysis, diagnosis, mutation, treatment, management and also all the factor deficiencies which are considered as RBD. Knowledge towards RBDs is increasing, however, most of published data are limited to small group of populations or case reports. Therefore, there are still several questions on these rare disorders which need to be clarified through large prospective studies.
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نویسندگان مقاله مجید نادری | majid naderi
departement of pediatrics hematology amp;amp; oncology, ali ebn-e abitaleb hospital research center for children and adolescents health [rccah], zahedan university of medical sciences, zahedan, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی زاهدان (Zahedan university of medical sciences)

شادی طبیبیان | shadi tabibian
department of hematology, allied medical school, tehran university of medical sciences, tehran, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

مریم سادات حسینی | maryam sadat hosseini
department of hematology, allied medical school, tehran university of medical sciences, tehran, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

شعبان علیزاده | shaban alizadeh
department of hematology, allied medical school, tehran university of medical sciences, tehran, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

سودابه حسینی | soudabeh hosseini
department of hematology, allied medical school, iran university of medical sciences, tehran, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی ایران (Iran university of medical sciences)

حسین کرمی | hossein karami
thalassemia research center, mazandaran university of medical sciences, sari, iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی مازندران (Mazandaran university of medical sciences)

حسن محمودی نشلی | hassan mahmoodi nesheli
non- communicable pediatric disease research center, babol university of medical sciences, babol, iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی بابل (Babol university of medical sciences)

اکبر درگلاله | akbar dorgalaleh
department of hematology, allied medical school, tehran university of medical sciences, tehran, ir iran

سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

نشانی اینترنتی http://jpr.mazums.ac.ir/browse.php?a_code=A-10-255-1&slc_lang=en&sid=en
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زبان مقاله منتشر شده en
موضوعات مقاله منتشر شده ژنتیک پزشکی
نوع مقاله منتشر شده مرور سیستماتیک
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