Prophylaxis in Hemophilia
نویسندگان
چکیده
Hemophilia is an inherited X-linked coagulopathy defined by a deficiency or abnormality in the clotting function of factor VIII (Hemophilia A) IX B). Prophylaxis – regular administration therapeutic products to maintain hemostasis and prevent bleeding mainstream treatment. Addressing development scientific evidence for administrating prophylaxis goal this review. modality choice people with severe hemophilia, being considered, principle, lifelong It should have early onset, ideally as primary, at least secondary. Even tertiary seems offer benefit, although further studies are still lacking. Individualized strategies lead optimization dilemma between better joint outcomes versus involved costs.
منابع مشابه
Optimization of prophylaxis for hemophilia A
BACKGROUND & AIMS Prophylactic injections of factor VIII reduce the incidence of bleeds and slow the development of joint damage in people with hemophilia. The aim of this study was to identify optimal person-specific prophylaxis regimens for children with hemophilia A. METHODS Analytic and numerical methods were used to identify prophylaxis regimens which maximize the time for which plasma f...
متن کاملTailored prophylaxis in severe hemophilia A: interim results from the first 5 years of the Canadian Hemophilia Primary Prophylaxis Study.
BACKGROUND Prophylactic treatment for severe hemophilia A is likely to be more effective than treatment when bleeding occurs, however, prophylaxis is costly. We studied an inception cohort of 25 boys using a tailored prophylaxis approach to see if clotting factor use could be reduced with acceptable outcomes. METHODS Ten Canadian centers enrolled subjects in this 5-year study. Children were f...
متن کاملEffects of coagulation factor concentrate prophylaxis in moderate and severe hemophilia A patients at a single hemophilia center in Korea
PURPOSE The aim of this study was to investigate prophylactic treatment effects in Korean patients with severe hemophilia A. METHODS A prospective study of 32 severe hemophilia A patients was conducted with the approval of the Institutional Review Board at the Eulji University Hospital. Two patients received primary prophylaxis; whereas, the other 30 patients were divided into 2 groups-second...
متن کاملAthletic participation in severe hemophilia: bleeding and joint outcomes in children on prophylaxis.
OBJECTIVES We sought to determine joint outcomes relative to impact level of athletic participation among school-aged children who had hemophilia and were taking prophylactic factor replacement, as well as to investigate prognostic factors for joint outcomes. METHODS School-aged boys with severe hemophilia A or B at a single center were included in the study. Clinical data on baseline joint s...
متن کاملRecent Advances in Hemophilia Gene Therapy
Heterogeneous loss of function mutations at F8 and F9 genes causes X-linked recessive bleeding disorders, hemophilia A (HA) and hemophilia B (HB), respectively. HA is clinically indistinguishable from HB and accounts for more than 80% of hemophilia cases; the former affects 1/5000 and the latter 1/25000 male births worldwide. In Iran, it is estimated that around 4300 HA and 900 HB patients are ...
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ژورنال
عنوان ژورنال: Clinical & Biomedical Research
سال: 2023
ISSN: ['2357-9730']
DOI: https://doi.org/10.22491/2357-9730.126436