HEMOPHILIA

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Dr Ira Shah
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Other Complications
Most hemophiliacs treated with concentrates before 1986 have been tested to be HIV positive. Factor concentrates are now subjected to screening and viral inactivation to eliminate HIV transmission. With proper viral inactivation methods, the risk of Hepatitis B and C has already decreased. Immunization with Hepatitis B vaccine has eliminated the risk of hepatitis B.

Joint Protection in Hemophiliacs
In hemophiliacs, joint bleeding and re-bleeding is common. Bleeding into joints cause inflammation of the synovium. With repeated hemorrhages, chronic synovitis may ensue leading to proliferation of the inflamed vascular synovial membrane causing rebleeding. Thus, a vicious cycle is created. Cartilage is gradually destroyed and bone is reabsorbed causing degenerative arthritis. Joint bleeding during growing years may cause excess growth at the ends of long bones leading to limb-length discrepancies. Hemorrhage into muscles may lead to atrophy and contracture.

Chronic synovitis - It presents with effusion commonly in the knee causing a swollen, boggy and warm joint. Concentrate prophylaxis for 2-4 weeks plus 1-2 weeks course of oral prednisone (0.5 mg/kg/d) should be tried. While concentrate is being given, the strength of the adjacent muscles should be improved with isometric and other exercises. Posterior splints should be worn at night to avoid injury during sleep.

It the above measures fail, synovectomy should be considered.

Flexion contractures - it is commonly seen at the elbow and the knee. At the elbow, an enlarged radial head often impedes forearm rotation. Excision of the radial head may prove to be beneficial. Knee flexion contractures can be treated with cylinder casts around the thigh and calf.

Degenerative Arthritis - It is seen adults with severe hemophilia. They benefit from use of NSAIDS and weight reduction. If chronic disabling pain persists in shoulder, ankle or subtalar joint, joint fusion may give relief. Prosthetic knee, hip or shoulder replacement may be useful.



Contributor Information and Disclosures Dr Ira Shah
Consultant Pediatrician, B.J.Wadia Hospital for Children, Mumbai, India


First Created : 12/20/2000
References
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