Long term follow up study on a large group of patients with congenital factor XIII deficiency treated prophylactically with fibrogammin P®

Factor xiii deficiency (fxiiid) is an extremely rare hemorrhagic disorder with a prevalence of 1/3-5 million. management of disease is performed by fresh frozen plasma (ffp),cryoprecipitate (cp) or fxiii concentrate (fibrogammin p®). our objective was to assess safety and effectiveness of fibrogammin p® in patients with fxiiid. for this purpose we designed this long-term follow up study on a large group of patients with fxiiid. this prospective study was conducted on 213 patients with fxiiid since 2009 to 2013. administrated dose for fibrogammin p® according to clinical situations of patients ranged from 10 to 26 iu/kg every 4-6 weeks. all patients in 6-month intervals were checked for human immunodeficiency virus (hiv),hepatitis a,b and c viruses (hav,hbv,hcv). twelve percent of participants had at least one ich episode until 2008 but after administration of fibrogammin p® did not have any major bleeding or episode of ich,except in one patient. we also had 7 females with recurrent miscarriage that were managed successfully with a dose of 10 to 26 iu/kg every 4-6 weeks. this dose also was quite successful in management of major and minor surgery. none of the participants showed allergic reaction during treatment. a total of 7155450 iu of fibrogammin p® were infused but nobody was positive for hiv,hav,hbv,and hcv. we found that fibrogammin p® is a safe and effective therapeutic choice in management of fxiiid. © 2016 by school of pharmacy.