Efficacy of prophylaxis and genotype-phenotype correlation in patients with severe Factor X deficiency in Iran
- M. Karimi
- A. Vafafar
- S. Haghpanah
- M. Payandeh
- P. Eshghi
- H. Hoofar
- A. Afrasiabi
- J. Gerdabi
- R. Ardeshiri
- M. Menegatti
- F. Peyvandi
DOIAbstract
We aimed to evaluate the effect of regular prophylaxis with a Factor X (FX) concentrate for patients with severe FXD in Iran and to assess the correlation of the genotype and phenotype in these patients. Ten patients with severe FXD (FX activity <1%) were enrolled and characterized during 2010-2011. Prophylaxis with 20\u2003IU FX P Behring per kg body weight was administered once a week. FX levels, were monitored at baseline, 15 and 30\u2003min, 1, 3, 6, 12, 24, 48, 72 and 96\u2003h after starting prophylaxis. All patients were followed for 1\u2003year. The mean age of the patients was 15\u2003\ub1\u20037.8\u2003years (age range of: 6-27\u2003years). One patient had anaphylactic reaction after the first infusion, and the treatment was stopp...
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