Congenital Afibrinogenemia. Hereditary deficiency of other clotting factors
Conditions
Interventions
Infusion of Octafibrin.
Treatment - Drugs
Infusion of Octafibrin
Sponsors
Octapharma
Eligibility
Sex/Gender
All
Age
6 Years to 12 Years
Inclusion criteria
Inclusion criteria: Inclusion: age 6-12; documented diagnosis of congenital fibrinogen deficiency; Historical plasma fibrinogen activity of <50ml/dl; expected to have an acute bleeding episode(spontaneous or after trauma) or planing to undergo elective surgery; Informed consent signed by the subject's legal guardian Exclusion: Life expectancy<6 months; bleeding disorder other than congenital fibrinogen dificiency; prophylactic treatment with a fibrinogen concentrate; any fibrinogen concentrate or other fibrinogen-containing blood product within 2 weeks prior to start of treatment for the PK phase,a bleeding episode, or surgery; any coagulation drug within 1 week prior to start of PK phase or treatment for the bleeding episode or surgery, or as a planned or expected medication during the time period from Day 1 until 24 hourspresence or history of hypersensitivity to study medication,DVT or pulmonary embolism, arterial thrombosis within 1 year prior to start drug infusion,hypersensitivity to human plasma protein,Oesophageal varicose bleeding
Exclusion criteria
Exclusion criteria:
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Overall clinical assessment of the haemostatic efficacy of Octafibrin. Timepoint: 24 hours. Method of measurement: 4-point haemostatic efficacy scale. | — |
Secondary
| Measure | Time frame |
|---|---|
| ‘clot strength’ or ‘clot firmness’. Timepoint: 1 hour. Method of measurement: blood sampling. | — |
Countries
Iran (Islamic Republic of)
Contacts
Public ContactMehran Karimi
Shiraz University of Medical Sciences, Hematology Research Center
Outcome results
None listed