الفهرس 
HemophiliaOnly 14 pages are availabe for public view
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Abstract
Hemophilia is the most common recessive inherited x-linked bleeding disorder (Peyvandi et al., 2006). The most common type is factor-VIII deficiency (hemophilia A) that is also known as the classic type of hemophilia (Madhok et al., 1991). Hemophilia A is classified into three different degrees, which describe the disease severity. In the severe form the clotting factor is less than 1% of normal factor level in blood, in the moderate form it is between 1 and 5%, and the mild form it is between 5 and 40% of normal level (Madhok et al., 1991, Quinons et al., 2003 and Lundin et al., 2004).
The aim of our work is to find out the role of musculoskeletal ultrasonography as a tool for assessment of joint affection in hemophilic knee arthropathy compared to clinical, functional and routine x-ray.
Our study was carried out on 20 patients of type A hemophilic children with severe factor VIII deficiency aged from 6 to 15 years. The controls were 20 non-hemophilic children free from any joint affection. The controls were matched in age and sex with the cases. All participants were gathered from Physical Medicine, Rheumatology and Rehabilitation clinic in the Children’s hospital, Ain-Shams University.
All patients included in this study were subjected to:
1- Full history taking.
2- General clinical examination.
3- Local examination with clinical assessment by the Hemophilic Joint Health Score, the Total HJHS score ranged from 15 to 104 with mean of 64.9 ± 29.89.
4- Functional assessment according to Functional Independence Score for Hemophilia ranged from 14 to 31 with mean of 23.35 ± 5.13.
5- Laboratory investigations.
6- Radiological evaluation that was performed by the conventional X-ray according to (Pettersson classification) which ranged from 0 to 13 with mean of 4.30 ± 3.09, and the ultrasongraphic scoring according to Klukowska et al., 2001 and Muca-perja et al., 2012 as well as a quantitative scoring for cartilage thickness and knee joint space.
The results obtained were statistically analyzed and summarized as follows:
• All the hemophilic patients were of the severe form of hemophilia type A with hemophilic joint arthropathy.
• 40% of the patients were treated by Factor VIII replacement whether as a prophylactic or on demand, while 60% were treated by cryoprecipitate plasma regularly as a prophylactic or on demand as well.
• According to the patients’ radiological scoring, it has a highly significant positive correlation with age and the clinical scoring with P value < 0.01, while a significant negative correlation with their functional scoring with P value 0.03.
• The prevalence of cartilage damage among patients according to Klukowska et al., score was 75% of the total 40 knee joints, 47.5 % for synovial hypertrophy, 30% for synovial fluid and 20% for synovial vascularity.
• The prevalence of cartilage damage among patients according to Muca-perja et al., 2012 was 90% out of the total 40 knee joints, 47.5% for synovial hypertrophy, 27.5% for bone erosions and 20% for synovial hyperemia.
• On correlating the cartilage thickness damage of the knee joint among patients with the synovial hypertrophy, a significant statistically positive relation with P value was 0.011.
• The ultrasonography qualitative scores Klukowska et al., score and Muca-perja et al., 2012 on correlating with clinical, functional and radiological scores, no statistically significant relation was detected.
• On comparing the cartilage thickness over the hyaline cartilage of the knee joint between patients and controls, a highly statistically significant difference was detected. As well as, on comparing the joint space in the knee joint between the patients and the controls, a highly significant difference was detected.
• On comparing the cartilage thickness over the bony surfaces on the elbow joint (humerus and radius bones), and on the ankle joint (the tibia and talus bones) between patients and controls, a significant statistical difference was detected over the talus bone.
• On correlating the cartilage thickness over the hyaline cartilage among patients, as well as the joint space, a highly significant negative relations with age, clinical scores and the radiological score.
• On correlating the cartilage thickness over the hyaline cartilage among patients, as well as the joint space, no statistical significance relation was detected with the functional scoring.
• The patients were classified according to their cartilage thickness thinning and knee joint space narrowing compared with the controls’ knee cartilage thickness and joint space findings into a cut off points. The severe cartilage thinning were 47.5% among patients on their medial condyles, 37.5% were laterally and 45% were at their sulcus cartilage.
• The severe joint space narrowing according to the cut off points among patients were 42.5% at lateral side and 40% on the medial side of their knee joint.
• The knee joint was the predominant joint affected among the hemophilic patients more than their ankle or elbow joints. The cartilage thinning was the predominant pathological disorder especially at the medial condylar side.