congenital hemophilia A/B with inhibitors. Hereditary factor VIII deficiency, Hereditary factor IX deficiency
Conditions
Interventions
Intervention 1: in interventional group recombinant factor VIIa (Aryogen) is injected to patients at below style:90µ g/kg as start dose then 120µ g/kg every 2 hours for 3 dose. Intervention 2: in cont
Treatment - Drugs
in interventional group recombinant factor VIIa (Aryogen) is injected to patients at below style:90µ g/kg as start dose then 120µ g/kg every 2 hours for 3 dose
in control group Novoseven® is injected to patients at below style: 90µ g/kg as start dose then 120µ g/kg every 2 hours for 3 dose
Sponsors
AryoGen Biopharma Company
Eligibility
Sex/Gender
All
Age
2 Years to 80 Years
Inclusion criteria
Inclusion criteria: Inclusion criteria: known case of congenital hemophilia A/B with inhibitors level more than 5 Bethesda unit, age older than 2 , gender male or female, proper IV line for drug injection, the severity of bleeding should be evaluable Exclusion criteria: Presence of any congenital or acquired coagulopathy disorders except for congenital hemophilia A/B with inhibitors such as:( liver disease (hepatitis), uremia, malignancy, vitamin K deficiency) , severe atherosclerotic disease, any local pathology that predisposes the patient to bleeding, having been under immune tolerance induction therapy during one month prior to the study, Platelet count lower than 50000
Exclusion criteria
Exclusion criteria:
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Pain intensity. Timepoint: 1,3,6,9 hours after first injection(according to Kavakli global response scoring system). Method of measurement: clinically,by the hematologist(according to Kavakli global response scoring system).;Joint mobility. Timepoint: 1,3,6,9 hours after first injection(according to Kavakli global response scoring system). Method of measurement: clinically,by the hematologist detection (according to Kavakli global response scoring system). | — |
Secondary
| Measure | Time frame |
|---|---|
| Serum level of factor VIIa (FVII:C). Timepoint: in time zero(immediately befor injection) and 20 minutes after first injection. Method of measurement: by ELISA labratory test.;Amplitude of probable side effects. Timepoint: during therapy and monthly until 3 months after therapy. Method of measurement: clinically, by the hematologist. | — |
Countries
Iran (Islamic Republic of)
Contacts
Public ContactDr. Kamran Kamyar
AryoGen Biopharma Company
Outcome results
None listed