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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 Fibrogamm...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Shaheed Beheshti University of Medical Sciences
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018293/ https://www.ncbi.nlm.nih.gov/pubmed/27642336 |
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author | Naderi, Majid Karimi, Mehran Hosseini, Maryam Sadat Moradi, Es′hagh Shamsizadeh, Morteza Dorgalaleh, Akbar |
author_facet | Naderi, Majid Karimi, Mehran Hosseini, Maryam Sadat Moradi, Es′hagh Shamsizadeh, Morteza Dorgalaleh, Akbar |
author_sort | Naderi, Majid |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5018293 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Shaheed Beheshti University of Medical Sciences |
record_format | MEDLINE/PubMed |
spelling | pubmed-50182932016-09-16 Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® Naderi, Majid Karimi, Mehran Hosseini, Maryam Sadat Moradi, Es′hagh Shamsizadeh, Morteza Dorgalaleh, Akbar Iran J Pharm Res Original Article 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. Shaheed Beheshti University of Medical Sciences 2016 /pmc/articles/PMC5018293/ /pubmed/27642336 Text en © 2016 by School of Pharmacy Shaheed Beheshti University of Medical Sciences and Health Services |
spellingShingle | Original Article Naderi, Majid Karimi, Mehran Hosseini, Maryam Sadat Moradi, Es′hagh Shamsizadeh, Morteza Dorgalaleh, Akbar Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title | Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title_full | Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title_fullStr | Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title_full_unstemmed | Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title_short | Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® |
title_sort | long term follow up study on a large group of patients with congenital factor xiii deficiency treated prophylactically with fibrogammin p® |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018293/ https://www.ncbi.nlm.nih.gov/pubmed/27642336 |
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