Haemophilic arthropathy.
نویسندگان
چکیده
Haemophilia is a heredity disorder in which bleeding is due to deficiency of coagulation factor VIII. Haemophilia A is the second most common inherited coagulation disorder. Estimation of its incidence ranges from 1 in 20,000 to as high as 1 in 10,000 people. It is a classic example of x-linked recessive trait (1). The frequency and severity generally are related to the blood level of factor VIII C (2). Those with less than 1% of normal activity develop severe disease, levels between 2% and 5% of normal are associated with moderate disease and patient with 6-50% of activity develop mild disease (3). In haemophilia, arthropathy is secondary to recurrent halmarthrosis and chronic synovitis (4). The chronic arthropathy affects only a few joints, most notably the knees and elbows and involvement of other joints like shoulders, hips and ankles is less common(5). The ankle joint causes the maximum limitation of motion, even though synovitis caused by it is the least common 2.2%(6).While advent of replacement therapy has dramatically changed the course of treatment and prognosis for patients with haemophilia, authors argue that economic burden of treating these patients is still very high (7). We also present a case of Abstract Bleeding into joints and soft tissues are the hallmark of haemophilia. The severity of manifestations co-relates with deficiency of the factor. The resorption of intra-articular blood induces reactive synovitis and causes cartilage damage, finally causing complete destruction of it. We hereby report a similar case and highlight various rheumatic manifestations of haemophilia and its management.
منابع مشابه
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ورودعنوان ژورنال:
- Indian pediatrics
دوره 6 1 شماره
صفحات -
تاریخ انتشار 1969