Prophylactic therapy with Fibrogammin P is associated with a decreased incidence of bleeding episodes: a retrospective study.
نویسندگان
چکیده
SUMMARY Congenital factor XIII (FXIII) deficiency is an extremely rare, yet potentially life-threatening, bleeding disorder, with a 30% rate of spontaneous intracranial haemorrhage. Routine prophylactic management is recommended for all individuals with clinically relevant (FXIII) deficiency and for all symptomatic individuals with congenital factor deficiency. Fibrogammin P is a purified, pasteurized concentrate of FXIII that appears to carry negligible risk of viral transmission, unlike other unprocessed products containing FXIII. An ongoing Phase II/III study of Fibrogammin P in patients with congenital FXIII deficiency is being conducted to evaluate the prophylactic efficacy and long-term safety of this product. Using retrospective chart review data from subjects enrolled in the Phase II/III study, the current analysis was designed to compare spontaneous bleed-event rates prior to and after the initiation of Fibrogammin P prophylaxis. Seven subjects were evaluable for comparison, having received no other prophylactic FXIII-containing product during the 24 months prior to study entry. The mean annual number of spontaneous bleeds was 2.5 events per year prior to Fibrogammin P prophylaxis and 0.2 events per year during Fibrogammin P prophylaxis (P = 0.01). Patients reported no severe bleeds during Fibrogammin P therapy. This small sample supports a consistent and clinically meaningful reduction in spontaneous bleeding with prophylactic use of Fibrogammin P.
منابع مشابه
Current understanding in diagnosis and management of factor XIII deficiency
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...
متن کاملLong 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 l...
متن کاملLong 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 l...
متن کاملMaintenance Therapy by Vaginal Progesterone after Threatened Idiopathic Preterm Labor: A Randomized Placebo-Controlled Double-blind Trial
Background Patients with arrested preterm labor (PTL) are at increased risk for recurrence of preterm birth (PTB). Maintenance tocolysis after arrest of acute PTL is of questionable value. The objective of this study was to evaluate the efficacy of 200 mg vaginal progesterone in order to prevent PTB in women with episodes of threatened PTL. MaterialsAndMethods This is a randomized double blind ...
متن کاملAdverse reactions associated with intravenous immunoglobulin therapy in patients with dermatologic diseases: An 11-year retrospective study
Background: Intravenous immunoglobulin (IVIG) is used to treat many autoimmune and immunodeficiency disorders. The main indications of IVIG in dermatology include treatment for resistant autoimmune bullous diseases, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). Although generally welltolerated, various adverse effects can occur.Methods: We designed a retrospective study ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haemophilia : the official journal of the World Federation of Hemophilia
دوره 16 2 شماره
صفحات -
تاریخ انتشار 2010