The efficacy of prophylactic treatment with coagulation factores in reducing the frequency of joint/non-joint bleedingand the consumption of coagulation factor in under 15 year old patients with severe A hemophilia

To evaluate the efficacy of preventive treatment with coagulation factors on number of joint/ non-joint bleeding and the consumption of coagulation factor

In this research, 12 patients with severe A hemophilia referring to comprehensive Hemophilia clinic of Imam Khomeini Hospital Complex will be selected purposefully. The participants will be selected successively and sampling will continue to reach the desired number.

First, an inhibitor level test will perform. In the cases have negative inhibitor level, a prophylactic treatment with factor VIII (at least 20 units per kg once a week and maximum 30 units per kg, three times per week) based on the severity of joint destruction and repetitive joint bleeding will be done. Study will be performed at comprehensive Hemophilia clinic of Imam Khomeini Hospital Complex. The study is not blind.

Inclusion criteria: Negative antibody level; Factor VIII less than 1% Exclusion criteria: Age over 15 years; Presence of an inhibitor in blood

First group (No bleeding in soft/articular tissue): No action The second group (Patients who have had at least one bleeding in soft or articular tissue that has not received a coagulation factor till now): Start dose: 25 units per kg once a week, first stage of positive change: 25 units per kg twice a week, second stage of positive change: 25 units per kg three times a week The third group (Patients who have surely received more than 50 days of coagulation factor): Start dose: 25 units per kg once a week, first stage of positive change: 25 units per kg twice a week, second stage of positive change: 25 units per kg three times a week The fourth group (Patients who have, or have probably, received less than 50 days of coagulation factor): Start dose: 50 units per kg once a week, first stage of positive change: 30 units per kg twice a week, second stage of positive change: 25 units per kg three times a week The program continues at start dose and the change to the first and second stages depends on the occurrence of one of the following conditions: • Incidence of 3 joint bleeding during the last three months of current prevention program • Incidence of 4 major soft tissue bleeding during the last three months of current prevention program • The occurrence of a dangerous and important bleeding without trauma or side effect despite of current prevention program. In other words, dose changes from the start dose to the first stage, and from the first to the second stage, occurs only in one of these situations.

the frequency of joint/ non-joint bleeding; the consumption of coagulation factors

Tehran University of Medical Sciences

The main outcomes' data, including testing, interventions and results of intervention will be shared .

Access starts after the publication of the results.

Researchers of academic institutions

Presenting a Introduction letter from the University

E-mail: dr.karimi70@gmail.com Dr. Katayun Karimi

After receiving the request and the introduction letter, Tehran University of Medical Sciences will get permission and the file will be sent to them.