الفهرس 
HemophiliaOnly 14 pages are availabe for public view
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Abstract
Back ground: Repeated bleeding into the joints leads to the development of chronic and progressive joint disease in 90% of patients with severe hemophilia. Owing to recurrent hemarthrosis, musculoskeletal outcome remains an important hallmark of treatment efficacy and prognosis in hemophilia. In addition to hemoarthrosis, Muscle bleed in hemophiliacs may occur in and might result in compressive neuropathy and may further contribute to physical disability in addition to hemarthrosis as the main contributor.
Aim of work: Evaluation of nerve and muscle disease in hemophilic children and adolescents and their contribution to physical disability.
Patients and Methods: This was a cross sectional study conducted on 20 hemophilic children and adolescents with hemophilic knee arthropathy (19 with hemophilia A and one with hemophilia B); following up in Hematology, Oncology Clinic, Children Hospital, Ain Shams University. Patients were assessed for physical joint disability using Hemophilia Joint Health Score (HJHS) of individual knee joints with scoring from 0 - 20 with 0 coding for normal joint function and 20 for the worst joint handicap, surface EMG of knee muscles and nerve conduction studies of both femoral and posterior popliteal nerves. 20 healthy subjects, served as the control group.
Results: All studied cases were males with mean age of 8.05±4.02 (range 5- 15) years. 11 patients had severe, 8 had moderate while only 1 had mild hemophilia. Mean HJHS for the studied individual knee joints was 7.4±6.6 (range 1- 20). There was statistically significant lower SEMG activity of quadriceps muscles in hemophilic patients compared to controls (mean 148.8, 335.5 ms/s; p 0.000) and in severe compared to mild/moderate hemophilia (mean 107.3, 198 respectively; p 0.03). There was statistically significantly lower femoral nerve amplitude and significantly lower femoral nerve conduction velocity in patients compared to control subjects with p value < 0.05.Also there was statistically significant delay in femoral nerve conduction velocity in patients with sever hemophilia compared with mild/ moderate hemophilia with p value 0.003. Significant negative correlation was found between HJHS of knee joint and each of femoral nerve conduction velocity (r -0.615), posterior tibial nerve conduction velocity (r -0.535) and SEMG of both quadriceps (r -.480) and hamstring muscles (r -.0.49).
In conclusion: Abnormal muscle and nerve functions are associated with hemophilic arthropathy and are likely to contribute to physical disabilities in hemophilic patients. Assessment of both by nerve conduction studies and SEMG are important in individualizing physical/ rehabilitation therapy in hemophilic arthropathy.