الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Inherited deficiencies of coagulation factors generally lead to lifelong bleeding, whose severity is inversely proportional to the degree of factor deficiency (less factor/more bleeding). Hemophilia A and B and von Willebrand disease (VWD) are the most common inherited coagulopathies. Other coagulopathies are rare which are characterized by deficiency of coagulation factors other than VIII and IX. (Peyvandi et al,2006).
90% of bleeding episodes involve the joints. Recurrent clinical and subclinical joint bleeding is the hallmark of hemophilia, which almost inevitably leads to arthropathy and loss of normal function .The first bleeding episodes usually involves knee, ankle and elbow joints. (Carlos et al, 2015).
Hemarthrosis tends to occur in spite of the synovial membrane’s capacity to reabsorb the blood. When there is a lot of blood in the joint, the synovial membrane becomes hypertrophied. Then, the hypertrophic synovium is more prone to bleed again, ending up in a vicious circle of hemarthrosis-synovitis-hemarthrosis. In addition, the blood in the joint can directly cause death of the chondrocytes in the joint cartilage. This leads to joint damage, which progresses within only a few years to destruction of the joint (Muc et al, 2012).
Ultrasonography (US) is intrinsically more flexible and time- and cost-effective than magnetic resonance imaging (MRI) as a screening tool. The main issue is how best to quantify the information generated at US in a simplified and quick method for evaluating and scoring disease activity and damage in hemophilic arthropathy with the final aim to integrate US in the routine practice of hemophilia centres as a close complement of physical examination (Carlo Martinoli et al, 2013).
Our study included 35 patients; 25 males, 10 females with age ranging from 6 months to 13 years (mean age 5.4 years), diagnosed with inherited coagulopathies. Our cohort included 14 patients diagnosed with Hemophilia A (HA), 9 patients with Hemophilia B (HB), 6 patients with VWD and 6 patients with rare coagulopathies ( 2 patients with F VII deficiency , 2 patients with F XIII deficiency , 1 patient with F I deficiency and 1 patient with F V deficiency).
Three comprehensive and evidence-based US scanning procedures to image the elbow, knee and ankle are established with the aim to increase sensitivity in detection of early signs of joint involvement while keeping the technique easy and quick to perform. Each procedure includes systematic evaluation of synovial recesses and selection of a single osteochondral surface for assessment of cartilage and bone damage. then, a simplified scoring system of Carlo Martinoli et al, 2013, based on an additive scale is created to define the joint status offering a simple tool to evaluate hemophilic arthropathy.
We found that;
The weight bearing joints are the most frequent affected joint in these patients ( 55 % of examined knee joints showed signs of hemophilic arthropathy, the affected ankles accounted for 44 % and the affected elbows accounted for 35 %).
The most frequent abnormality detected by ultrasound is synovial hypertrophy [90.5% of the sonographically affected knee joints, 92% of sonographically affected elbow joints and 88% of sonographically affected ankle joints .
We found that the correlation between the ultrasound scores of the assessed disease parameters and the severity of the disease was statistically significant.
The signs of hemophilic arthropathy regarding synovial hypertrophy, cartilage abnormality and bone damage were more noted in older children.