Prevention of the Musculoskeletal Complications of Hemophilia
نویسنده
چکیده
Hemophilia is an inherited disorder of clotting factor deficiencies resulting in musculoskeletal bleeding, including hemarthroses, leading to musculoskeletal complications. The articular problems of hemophiliac patients begin in infancy. These include: recurrent hemarthroses, chronic synovitis, flexion deformities, hypertrophy of the growth epiphyses, damage to the articular cartilage, and hemophilic arthropathy. The most commonly affected joints are the ankle, the knee, and the elbow. Hematologic prophylactic treatment from ages 2 to 18 years could avoid the development of hemophilic arthropathy if the concentration of the patient's deficient factor is prevented from falling below 1% of normal. Hemarthroses can be prevented by the administration of clotting factor concentrates (prophylaxis). However, high costs and the need for venous access devices in younger children continue to complicate recommendations for universal prophylaxis. Prevention of joint arthropathy needs to focus on prevention of hemarthroses through prophylaxis, identifying early joint disease through the optimal use of cost-effective imaging modalities and the validation of serological markers of joint arthropathy. Screening for effects on bone health and optimal management of pain to improve quality of life are, likewise, important issues. Major hemarthrosis and chronic hemophilic synovitis should be treated aggressively to prevent hemophilic arthropathy.
منابع مشابه
Musculoskeletal manifestations of hemophilia: imaging features.
The purpose of this pictorial assay is to demonstrate the imaging features of the various musculoskeletal manifestations of hemophilia, an X-linked disorder. Depending on the site of recurrent bleeding, musculoskeletal manifestation can be in the form of hemophilic arthropathy and/or soft tissue, intraosseous, or subperiosteal pseudotumors. Radiography, sonography, computed tomography, and espe...
متن کاملRecent Advances in Hemophilia Gene Therapy
Heterogeneous loss of function mutations at F8 and F9 genes causes X-linked recessive bleeding disorders, hemophilia A (HA) and hemophilia B (HB), respectively. HA is clinically indistinguishable from HB and accounts for more than 80% of hemophilia cases; the former affects 1/5000 and the latter 1/25000 male births worldwide. In Iran, it is estimated that around 4300 HA and 900 HB patients are ...
متن کاملErgonomics: A solution for Prevention of musculoskeletal disorders in Intensive Care Unit nursing staff
Introduction:: Today, musculoskeletal disorders are one of the biggest health problems in the world. Prevention of musculoskeletal complications in ICU staff due to inappropriate physical posture at work, is important. The aim of the study is to assess the effect of education on musculoskeletal disorders in ICU staff nurses. methods: This interventional study performed on 90 nurses in ICU of ed...
متن کاملWhat´s New in Orthopedic Surgery for People with Hemophilia
Modern orthopedic surgery can improve the musculoskeletal problems of people with hemophilia. The most commonly affected joints are the knees, the ankles and the elbows. The most common orthopedic procedures that PWH undergo are the following: RS, arthroscopic synovectomy, arthroscopic joint debridement, ankle arthrodesis, total joint arthroplasty and removal of pseudotumors. Every surgical pro...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012