title: Iranian Low-dose Escalating Prophylaxis Regimen in Children with Severe Hemophilia A and B.
abstract: Establishing an appropriate prophylaxis regimen for children with hemophilia is a critical challenge in developing countries. Barriers including availability and affordability, catheter-related complications, and inhibitor development risks have led to the introduction of new tailored prophylaxis regimens in different countries. This study emphasizes on the benefits of the Iranian low-dose escalating prophylaxis regimen in a Hemophilia Comprehensive Care Center in Iran. Referred patients with hemophilia less than 15 years of age, who were subject to prophylaxis regimen, are studied retrospectively. A once-weekly prophylaxis regimen of 25 IU/kg was started for the patients primarily. Their prophylaxis regimen was changed to 25 IU/kg twice a week and then 3 times a week when they experienced 3 joint bleedings, 4 soft tissue bleedings, or a 1 life-threatening bleed without a specific trauma history. Overall, 25 patients with severe hemophilia and at least 6-month history of on-demand (OD) treatment were studied. A mean of 1754 IU/kg/yr of coagulation factors, used for OD and prophylaxis purposes, was sufficient to decrease the mean annual bleeding rate (ABR) to 1.86 after prophylaxis. It also reduced the mean hospitalization days and the mean number of target joints to 0.24 and 0.16, respectively. Overall, 19 (76%) patients were continuing their once-weekly regimen at the end of the follow-up. None of the patients needed 3-times-a-week regimen or central venous catheterization and none developed inhibitors in the follow-up. Benefits of the Iranian low-dose escalating prophylaxis regimen prove equal to some of the previous 3-times-a-week prophylaxis regimens in reducing the ABR and hospitalizations.
authors.affiliation: 1 Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Peyman
authors.initials: P
authors.lastname: Eshghi
authors.affiliation: 1 Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Elham
authors.initials: E
authors.lastname: Sadeghi
authors.affiliation: 1 Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: S Zahra
authors.initials: SZ
authors.lastname: Tara
authors.affiliation: 1 Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Behnaz
authors.initials: B
authors.lastname: Habibpanah
authors.affiliation: 2 Department of Transplantation and Special Diseases, Ministry of Health and Medical Education, Tehran, Iran.
authors.forename: Razieh
authors.initials: R
authors.lastname: Hantooshzadeh
mesh_terms: D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000517:prevention & control; D002836:Hemophilia B / Q000188:drug therapy* / Q000517:prevention & control; D006470:Hemorrhage / Q000188:drug therapy / Q000517:prevention & control; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007492:Iran; D008297:Male; D011292:Premedication / Q000379:methods*; D012189:Retrospective Studies
chemical_list: D001779:Blood Coagulation Factors
keywords: hemophilia; low dose; prophylaxis