[French guidelines. Long-term prophylaxis for severe haemophilia A and B children to prevent haemophiliac arthropathy].

During the last decades, long-term prophylaxis has become the gold standard for the treatment of children with severe haemophilia A or B. Prophylactic replacement regimens modify the natural history of the disease by aiming at the prevention of haemarthrosis, target joints and arthropathy. This treatment represents a constraint and an enhanced exposure to anti-haemophilic concentrates, which means potential increase of related risks and significant additional cost.

Epidemiological survey of haemophiliacs with inhibitors in France: orthopaedic status, quality of life and cost--the 'Statut Orthopédique des Patients Hémophiles' avec Inhibiteur study.

The physical condition of severe haemophilia and the impact of advances in replacement therapy have been much studied, but little work has been done on patients who developed inhibitors. The 'Statut Orthopédique des Patients Hémophiles avec Inhibiteur' study was conducted in France in order to assess the orthopaedic status and quality of life of such patients, and the cost of their medical management. Fifty haemophiliacs aged 12-63 years with a history of high-responder inhibitors were included.

The role of the physiatrist in the haemophilia comprehensive care team in different parts of the world.

The role of the physiatrist, as a member of the multidisciplinary haemophilia comprehensive care team, is to prevent and treat activity limitations and restriction of participation on the part of the patient. . This role is threefold: (i) provide education to the patients, families and healthcare providers to detect disabling injuries and take adequate precautions; (ii) provide specialized treatment of musculoskeletal disabilities striving for the highest level of functionality and (iii) Stimulate the patient's full participation in socio-economic activities of his country.

Management of haemophilic arthropathy.

Despite the tremendous benefit offered by primary prophylaxis, recurrent joint bleeding with progression to chronic synovitis and haemophilic arthropathy is still a daily concern for the multidisciplinary health care teams managing patients with severe haemophilia or haemophilia complicated by inhibitor development. Advanced stages of arthropathy could be prevented by regular assessment of musculoskeletal status and thus early detection of symptoms, daily rehabilitation exercises at home, and implementation of appropriate physiotherapy and medical training. Patient's education and psychological counselling are crucial.

Epidemiological survey of the orthopaedic status of severe haemophilia A and B patients in France. The French Study Group. secretariat.haemophiles@cch.ap-hop-paris.fr.

One hundred and 16 patients contributed to an analysis of the impact of the consequences of severe haemophilia A or B (factor levels < 2%) on orthopaedic status, resources consumed in relation to this status and resultant cost, and quality of life as perceived by the patient, using the MOS 36-Item-Short-Form Health Survey (SF-36). This French cross-sectional study involved outpatients regularly attending a haemophilia treatment centre. Data were collected retrospectively over a period of 1 year by the physician of the haemophilia treatment centre.

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.

MR imaging in hemophilic arthropathy.

20 children with hemophilia were examined with Magnetic Resonance Imaging (MRI) to determine if MRI could be used to assess hemophilic arthropathy, especially in the early stages. 28 joints of the appendicular skeleton were imaged, including 17 knees, 10 ankles and 1 elbow. The results were compared to clinical and plain radiographic assessments and to the surgical findings when synovectomy was performed.

Clinical and biological survey of haemophilia A and B patients infused with French heat-treated concentrates.

The efficiency of heat treatment procedures of factor VIII and factor IX concentrates, prepared from voluntary, non-paid donors by three French Blood Transfusion Centres, on the inactivation of HIV and non-A, non-B hepatitis (NANB) viruses was assessed. Some 43 patients (26 haemophilia A, 17 haemophilia B) were followed for at least 1 year by testing for HIV antibodies and alanine aminotransferase (ALT). No HIV seroconversion was observed indicating that heat treatment was completely efficient.

[Importance of synoviorthesis using osmic acid in the treatment of hemophilic arthropathy. Apropos of 19 cases].

While getting synovial sclerosis and articular drying, the synoviorthesis breaks the circulus vicious which comes from iterative hemarthrosis to articular destruction. With 25 cases and a follow up of 6 years, we have got 39% of good results, 35% of medium and 26% of bad ones. The therapeutic efficiency requires a certain latent time (0 to 15 days for isotopical synoviorthesis, 2 to 3 months for those ones with osmical acid.