Cases reported "Hemophilia A"

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1/10. elbow joint, crutches and locomotion: special reference to persons with haemophilia.

    A 52-year-old, trans-femoral amputee with haemophilia was hospitalized because of ambulatory problems arising from the osteo-arthropathic involvement of other major articulations. Reduced function in the upper limbs, caused by the effects of recurrent haemarthroses, resulted in additional problems concerning the usage of auxiliary ambulatory aids. The advantages and disadvantages of traditional and experimental crutches highlight the functional problems of ambulation in persons with concomitant upper limb pathologies.
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2/10. Avascular necrosis of the talus in children with haemophilia.

    patients who have haemophilia often present with joint problems. Haemarthrosis can occur spontaneously or after minor trauma. Later, degenerative changes can cause pain and loss of motion. Avascular necrosis of the talus has a high incidence after serious trauma. Furthermore, avascular necrosis of the talus has been associated with multiple systemic conditions. The association of haemophilia and avascular necrosis of the femoral head has been described. This study reports three cases (four ankles) where we suspect an avascular necrosis of the talus in children with haemophilia. To the authors' knowledge, the possible association of haemophilia and avascular necrosis of the talus has not yet been described in children.
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3/10. rehabilitation of patients with haemophilia after orthopaedic surgery: a case study.

    Preoperative and postoperative rehabilitation may be useful for improving the recovery of patients undergoing orthopaedic surgery, particularly in those with co-morbidity or special requirements. This case study, of a patient with haemophilia and inhibitors to factor viii undergoing total knee replacement, demonstrates the benefits of 6 weeks' preoperative physiotherapy ('prehabilitation') combined with 6 weeks' postoperative rehabilitation. The supervised physiotherapy regimen was individually tailored to specifically increase range of motion and muscle strength, enabling rapid mobilization and recovery of function, whilst minimizing the risk of bleeding.
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4/10. Operative treatment of anterior heterotopic bone formation of the elbow in a patient with severe haemophilia A.

    There is no statistical data on the incidence of heterotopic ossification among patients with haemophilia, and a few reports documenting this entity in haemophilia are available. Although post-traumatic heterotopic ossification about the elbow is a well-recognized complication, we are not aware of any previously reported case in haemophiliacs. An 8-year-old boy with severe haemophilia A presented with fixed elbow in 80 degrees of flexion. Radiographs disclosed a mature anterior heterotopic ossification in the form of complete ulnohumeral bony bridge. Surgical excision of the heterotopic bone was performed. A full elbow range of motion was obtained after long-term physiotherapy. We conclude that this surgery is safe and successful in haemophilic patients, if performed at the right time under optimal situation.
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5/10. Bruising and hemophilia: accident or child abuse?

    Recognition and prompt reporting of suspect child abuse or neglect is necessary to institute programs aimed at preventing further neglect, re-injury or possible death. The families of children with chronic medical conditions, such as hemophilia, may be affected by economic and emotional stresses which may be expressed as abuse or neglect. Because the manifestations of even slight trauma are so common in children with bleeding problems, the physician may not routinely inquire about the cause of injury, delays in seeking medical attention, or accident prevention efforts in the home. Early referral of children with hemophilia to a multidisciplinary team providing medical, psychological, and social care is recommended.
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6/10. Synovectomy and continuous passive motion (CPM) in hemophiliac patients.

    Synovectomy of the knee is a procedure that has been used traditionally for the management of arthropathies due to intraarticular bleeding diatheses. Although open synovectomy controls recurrent bleeding problems, the success of this procedure has been limited frequently by disabling stiffness of the joint. In an attempt to improve on the results of open synovectomy, arthroscopic synovectomy was combined with continuous passive motion (CPM) in a prospective surgical and rehabilitation program. Five male patients aged 10 to 35 years, with chronic and/or recurrent hemarthroses due to hemophilia, underwent arthroscopic synovectomy of the knee. CPM was begun in the recovery room and continued for 5-7 days. Each patient had mildly restricted range of motion (ROM) immediately postoperatively as compared with preoperative measurements. Both active and passive ROM improved rapidly with CPM and physical therapy. By 3-6 months postoperatively, all patients except one had achieved an active ROM greater than the preoperative range, and all showed a significant reduction in documented bleeding episodes. The combination of arthroscopic techniques, which result in minimal extraarticular trauma, and immediate mobilization using CPM has been successful in maximizing the benefits and minimizing the complications of synovectomy in the patient with hemorrhagic arthropathy.
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7/10. Hemophilic arthropathy resulting in a locked shoulder.

    A 14-year-old boy with severe hemophilia had a swollen immobile left shoulder joint. A roentgenogram showed a severely deformed humeral head that had interlocked onto the glenoid ring. After manipulation, the motion promptly returned. Incongruency of the shoulder in adolescence poses a serious therapeutic problem.
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8/10. Use of continuous passive slow motion in the postoperative rehabilitation of difficult pediatric knee and elbow problems.

    Continuous passive slow motion (CPSM) was used to aid postoperative rehabilitation in seven difficult pediatric knee and elbow problems. Four patients had joint erosion secondary to hemophilic arthropathy and three patients had joint irregularities associated with long-standing limitation of joint movement. CPSM was started soon after surgery at the slow speed of 1 revolution every 12.5 min. The early, slow mobilization did not cause pain and established an arc of joint motion before intraarticular adhesions became a problem. Compared with a similar group not using CPSM, our hemophilia patients did not require postoperative manipulation, had decreased hospitalization time, and gained a greater range of motion. The group with long-standing limited joint mobility exhibited maintenance and in most cases improved motion after surgery. Thus CPSM after surgery enhances the rehabilitation process of difficult pediatric knee and elbow problems.
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9/10. Hemophiliac knee: rehabilitation techniques.

    Hemophilia is an inherited bleeding disorder which produces its greatest morbidity in the musculoskeletal system. This article reviews current rehabilitation techniques for the hemophiliac knee--including restrictions of physical activity, external support, and corrective orthotic devices--reports our experience using aggressive strengthening techniques in 2 children, and compares the effectiveness of the 2 methods. It is the impression of the authors that strengthening, rather than immobilization, can bring about increased range of motion, increased strength, and decreased bleeding frequency.
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10/10. Open synovectomy for the prevention of recurrent hemarthrosis of the ankle in patients with hemophilia. A report of five cases with magnetic resonance imaging documentation.

    Hemophilic arthropathy is an incapacitating complication of severe hemophilia resulting from recurrent bleeding in the same joint. Open synovectomy has been used since 1969 to prevent recurrent hemarthrosis of target joints. Between 1988 and 1993 we performed open synovectomy of the ankle in five hemophiliacs aged 6 to 9 years with early-stage hemophilic arthropathy. magnetic resonance imaging proved very useful for evaluating the severity of joint damage, usually underestimated on plain radiographs; for determining the degree of synovial membrane hypertrophy, which is a critical factor in the decision to perform synovectomy; for planning the surgical procedure and for explaining treatment failures. A decrease in the frequency of hemarthrosis episodes occurred in all five ankles. A repeat synovectomy was needed in one case and in another patient the frequency of hemarthrosis episodes increased somewhat after the fourth year. There was no loss of range of motion. Our data suggest that open synovectomy is effective and safe for reducing the frequency of hemarthrosis and that magnetic resonance imaging should be routinely performed before the procedure.
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