Severe Congenital Deficiency of Factor XIII: A Brief Report of Morbidity and Mortality Rates of Severe Congenital Deficiency of Factor 13 in Iran

• Main Authors: Akbar Dorgalaleh, Samira Esmaeili Reykandeh, Moreza Shamsizadeh, Behnaz Tavasoli, Eshagh Moradi
• Format: Article
• Language: English
• Published: Bushehr University of Medical Sciences 2017-04-01
• Series: Iranian South Medical Journal
• Subjects: Factor XIII deficiency; clinical features; Morbidity; Mortality
• Online Access: http://ismj.bpums.ac.ir/browse.php?a_code=A-10-3-751&slc_lang=en&sid=1

Background: Factor XIII (FXIII) deficiency is a bleeding disorder and it inherited in an autosomal recessive manner. in areas where consanguineous marriage is common, it has high prevalence. Iran as a Middle East country comprise the high rate of consanguinity in nearly half of patients with severe congenital FXIII deficiency (FXIIID). The most common mutation in Iranian population is Trp187Arg. In these patients occure life- threatening bleeding including central nervous system (CNS) bleeding, umbilical cord bleeding and recurrent miscarriage that have high rate of morbidity and mortality. The aim of this study was to investigate morbidity and mortality rate among these patients and the reasons for it. Materials and Methods:In this systematic review we  studied all published paper in the field of FXIIID factor deficiency in Iran until 2015 via searching in databases and search engine such as Sciencedirect, scientific information database (SID), PubMed and Google scholar. Results: Among 308 patients with FXIIID, 108 cases had experienced CNS bleeding (CNSB) that in 23 cases had associated with rebleeding. in 72 patients have occured different types of neurological complications after  central nervous system bleeding. A total of 63 recurrent miscarriages were observed in 30 women and 21 deaths were registered due to umbilical cord bleeding or mucosal bleedings. Conclusion: Due to high rate of morbidity and mortality among patients with FXIIID, early diagnosis of disease, prophylaxis treatment and intensive health care should be considered among these patients.