الفهرس 
HemophiliaOnly 14 pages are availabe for public view
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Abstract
Background: Hemophilia A is an X-linked bleeding disorder. The characteristic bleeding phenotype in patients with severe hemophilia is recurrent spontaneous hemarthrosis with development of hemophilic arthropathy. Heterozygous female carriers with factor VIII (FVIII) activity level lower than 40% may be at risk for bleeding that is usually comparable to that seen in males with mild hemophilia.
Aim of the Work: To assess factor VIII level in mothers of known patients of hemophilia A correlating it with the frequency of clinically and radiologically documented arthropathy.
Patients and Methods: A cross sectional study included 40 mothers of patient with hemophilia. Studied participants were subjected to detailed analysis of bleeding history, six major joints range of motion (ROM) examination, functional independence score in hemophilia (FISH), FVIII level one stage assessment and magnetic resonant imaging (MRI) of the reported affected joints.
Results: Mean FVIII activity was 43.33 IU (± 22.79), ranging from FVIII (0 < 30 IU) in 13 (32.5%), FVIII (30 <60) in 18 (45.0%) and FVIII ≥ 60 IU in 9 (22.5%) among the studied participants; knees were the most affected joints with decreased mean ROM (±SD) in right and left knees of (123.25 (±12.06) and 120.00 (±15.81)) respectively.
Conclusion: Potential female hemophilia A carriers are at risk of spontaneous bleeding which may entail bleeding in the joints, causing early joint damage.