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Effects of the factor V G1691A mutation and the factor II G20210A variant on the clinical expression of severe hemophilia A in children – results of a multicenter study
- Source :
- Haematologica, Vol 92, Iss 7 (2007)
- Publication Year :
- 2007
- Publisher :
- Ferrata Storti Foundation, 2007.
-
Abstract
- The present multicenter cohort study of 107 pediatric PUPs was performed to determine whether the concomitant inheritance of the factor (F) V G1691A or the F II G20210A mutation influences the clinical expression of severe hemophilia A (HA). Carriers of the FV and FII mutations had a significantly lower annual bleeding frequency (ABF) than non-carriers (p=0.012). Joint damage (Pettersson score) was significantly less severe in patients with thrombophilia (p=0.022). A protective effect of thrombophilic risk factors was shown for ABF (OR [CIs]: 0.7[0.5–0.9]; p=0.02) and the severity of the hemophilic arthropathy (OR [CIs]: 0.06[0.01–0.3]; p=0.0009).
- Subjects :
- Diseases of the blood and blood-forming organs
RC633-647.5
Subjects
Details
- Language :
- English
- ISSN :
- 03906078 and 15928721
- Volume :
- 92
- Issue :
- 7
- Database :
- Directory of Open Access Journals
- Journal :
- Haematologica
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.90d90ab52b3f4a3ba5bbad9c87878ffa
- Document Type :
- article
- Full Text :
- https://doi.org/10.3324/haematol.11161