Central Diagnostic Laboratories, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
Expert Rev Hematol. 2021 May;14(5):467-472. doi: 10.1080/17474086.2021.1923476. Epub 2021 May 10.
: Rare bleeding disorders (RBDs) are a heterogeneous group of bleeding disorders. Consanguineous marriage is the favorite partnership in many communities among a population of more than a billion. Consanguineous marriages allow the defective recessive genes in RBDs to remain in the family. Clinical approach to RBDs is not as straightforward as typical bleeding disorders, which are secondary to scarcity and variation of RBDs. However, in many developing countries, the limitations of diagnostic facilities, the supply of therapeutic coagulation concentrates, health budgets, and optimal health-care infrastructures may intensify the clinical practice of RBDs.: The author addresses the role of consanguineous marriage in the incidence of RBDs and what preventive steps can be considered to minimize the incidence of RBDs. A literature review of PubMed and google Scholar was conducted in November of 2020 using the relevant keywords.: This review addresses the prevalence of consanguineous marriages in Middle Eastern countries and the various reasons behind the coexistence of consanguineous marriage with RBDs. Finally, prevention steps that may be considered by health-care systems to minimize the incidence of rare bleeding disorders have been discussed. Health-care systems should train partners with the intention of consanguineous marriage on the downside of consanguinity.
: 罕见出血性疾病（RBDs）是一组异质性的出血性疾病。在拥有超过 10 亿人口的许多社区中，近亲结婚是最受欢迎的婚姻形式。近亲结婚使得 RBDs 中缺陷的隐性基因在家庭中得以保留。与典型的出血性疾病相比，RBDs 的临床方法并不那么直接，后者是由于 RBDs 的稀缺性和变异性引起的。然而，在许多发展中国家，诊断设施的局限性、治疗性凝血浓缩物的供应、卫生预算以及最佳的医疗保健基础设施可能会加剧 RBDs 的临床实践。: 作者探讨了近亲结婚在 RBDs 发病中的作用，以及可以考虑哪些预防措施来最大限度地减少 RBDs 的发病率。作者于 2020 年 11 月在 PubMed 和谷歌学术上使用相关关键词进行了文献回顾。: 本综述讨论了近亲结婚在中东国家的流行情况，以及近亲结婚与 RBDs 共存的各种原因。最后，讨论了医疗保健系统可以考虑采取哪些预防措施来最大限度地减少罕见出血性疾病的发病率。医疗保健系统应该对有近亲结婚意向的伴侣进行培训，让他们了解近亲结婚的弊端。
