تغييرات راديولوژيك استخواني ، مفصلي و نسج نرمي در بيماران مبتلا به هموفيلي

Radiological Findings in Hemophilia : An Experience of Hemophilia Clinic

Background and Objectives: Hemophilia is a hereditary bleeding disorder. It is divided to hemophilia A, B and C depending on deficiency of clotting factors involved. Hemophilia A is due to deficiency of factor VIII, hemophilia B is result of factor IX deficiency and hemophilia C is due to factor XI deficiency. Genetic abnormality resides in chromosome X in 80% of the cases. The women are carriers only, while men experience full picture of the disease. Materials and Methods: In this study, experiments were carried out on 241 patients. All patients with coagulation deficiency who were referred to Shahid Gazi Hospital, were examined by imaging technique. In patients with joint hemorrhage, plain films were taken, however when extra joint hemorrhage were seen, CT scan or ulirasonography were techniques or choice. In some cases all of these techniques were performed. Results: We report radiological findings observed in 241 patients. Of these, 192 were hemophiliacs, 164 suffering from hemophilia A, and 28 From hemophilia B. Knee joint was involved in 160, ankle in 62, elbow in 28, wrist in 34, shoulder in 26 and hip in 29 patients. Pseudotumor was seen in 3 patients. Conclusion: Sometimes death may results from hemorrhage. Treatment is mandatory, but treatment with factor or frozen serum is very expensive. Because hemophilia is X linked, birth contral in this patients is very important. Although the prevention of child breang in carriers and patients seem to be difficult by instruction, instruction can be helpfuli in preventing birth of male infants.