title: Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P®.
abstract: 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.
authors.affiliation: Department Of Pediatrics Haematology and Oncology, Ali Ebn-e Abitaleb Hospital Research Center for Children and Adolescents Health [RCCAH], Zahedan University Of Medical Sciences, Zahedan, Iran.
authors.forename: Majid
authors.initials: M
authors.lastname: Naderi
authors.affiliation: Hematology Research Center,Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
authors.affiliation: Department of Hematology and Blood Transfusion, School of Allied Medical Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Maryam Sadat
authors.initials: MS
authors.lastname: Hosseini
authors.affiliation: Faculty Member at Education Development Center (EDC), Urmia University of Medical Sciences, Urmia, Iran.
authors.forename: Es Hagh
authors.initials: EH
authors.lastname: Moradi
authors.affiliation: School of Nursing and Midwifery, Shahroud University of Medical Sciences, Shahroud, Iran.
authors.forename: Morteza
authors.initials: M
authors.lastname: Shamsizadeh
authors.affiliation: Department of Hematology and Blood Transfusion, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Akbar
authors.initials: A
authors.lastname: Dorgalaleh
keywords: Effectiveness; Factor XIII deficiency; Fibrogammin P®; Safety