Haemophilia and joint disease: pathophysiology, evaluation, and management
نویسندگان
چکیده
In patients with haemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of haemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (haemophilic synovitis) that contribute to end-stage degeneration (haemophilic arthropathy); with pain and limitation of motion severely affecting patients' quality of life. If joint bleeding is not treated adequately, it tends to recur, resulting in a vicious cycle that must be broken to prevent the development of chronic synovitis and degenerative arthritis. Effective prevention and management of haemophilic arthropathy includes the use of early, aggressive prophylaxis with factor replacement therapies, as well as elective procedures, including restorative physical therapy, analgesia, aspiration, synovectomy, and orthopaedic surgery. Optimal treatment of patients with haemophilia requires a multidisciplinary team comprising a haematologist, physiotherapist, orthopaedic practitioner, rehabilitation physician, occupational therapist, psychologist, social workers, and nurses. Journal of Comorbidity 2011;1:51-59.
منابع مشابه
The 2000 United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) inhibitor guidelines.
The UKHCDO inhibitor guidelines address the diagnosis and management of patients with haemophilia A, haemophilia B and acquired haemophilia. Recommendations are based on best current practice as reflected in the published evidence base. Many current treatment strategies are based on uncontrolled observations highlighting the need for well designed controlled studies.
متن کاملModern management of haemophilic arthropathy.
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BACKGROUND Problems concerning treatment of patients with haemophilia are long-term and exist even in the present days. Thanks to interdisciplinary complex therapy the results of treatment are constantly better than many years ago. AIM The goal of this study is to analyse the current state of management of haemophilia and to suggest a comprehensive concept of rational and effective rehabilita...
متن کاملModern treatment of haemophilia.
Many rapid advances have been made in the diagnosis and therapy of haemophilia. Nevertheless, the condition still poses problems and challenges (e.g., joint disease, transfusion-transmitted diseases, inhibitors, provision of care in developing countries, and education and cost issues). WHO and the World Federation of Hemophilia held a joint meeting in Geneva, on 21-23 March 1994, to discuss and...
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عنوان ژورنال:
دوره 1 شماره
صفحات -
تاریخ انتشار 2011