3 groups of hemophilia patients - Those with > 20 bleeds into a joint, those with < 2 bleeds into a joint and those with no bleeds into a joint will be enrolled into the study

3 groups of hemophilia patients Group I - Those with > 20 bleeds into a joint, Group II - those with < 2 bleeds into a joint and Group III - those with no bleeds into a joint will be enrolled into the study

plasma samples for angiogenic and cartilage degradation markers

Hemophilia A

Hemophilia B

Hemarthroses

ultrasound will be performed in hemophilia boys with a history of 20 joint bleeds- Group I, less than 2 joint bleeds into a joint -Group II and no joint bleeds- Group III

ultrasound

Feinstein Institute of Medical Research Northwell Health

Ultrasonography in Hemophilic Joint Disease and Serum Markers

Ultrasonography in Hemophilic Joint Disease

Synovial changes on USG- PDS:
Scoring system for synovitis which has 2 components - joint effusion and synovial thickening: Joint effusion will be defined as a compressible anechoic intracapsular area and the amount of fluid will be semiquantitatively scored usinga previosuly described scoring system by Martinoli et al. Quantitative assessment of the power Doppler signal: Power Doppler signal will be assessed subjectively for degree of vascularity -Table 2, Table 2
Degree of Vascularity PDS Signal Score Normal or minimal No signal or local dark red 1 Mild hyperemia Dark red to red 2 Moderate hyperemia Red to orange 3 Marked hyperemia Orange to yellow 4

Blood will be collected on the smae day of the study to measure synovial angiogenesis markers- VEGF, MMP-9, SDF-1, HIF-1 , endothelial progenitor cells and hematopoietic progenitor cells

hemarthrosis/hypoxia

Ultrasonography in Hemophilic Joint Disease and Serum Markers

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