Abstract:We describe here the management of eleven patients with fracture neck of femur. Excepting one patient all had severe haemophilia A. Nine patients were less than 50 years of age. Eight out of eleven patients had fracture after trivial trauma. Nine patients had closed reduction and one patient open reduction. The patient with non union had a Valgus osteotomy. All fractures united. The average time to union was 11 weeks (range:8-16). We followed either a low dose intermittent or a low dose continuous infusion fac… Show more
“…Reduced bone density in individuals with haemophilia is multi-factorial and if occurs during childhood phase, it is a major risk factor for acquisition osteopenia or osteoporosis in later of life [22]. RBD in severe haemophilia A is a concept which has been supported by several authors [3-5, 7, 11-18].…”
Section: Pathogenesis Of Reduced Bone Densitymentioning
In recent decays, quality of life and life expectancy of individuals with severe haemophilia A and B have been improved by better quality in treatment and more availability of blood coagulation products. Recently, new concerns have emerged in current groups such as auto-antibody inhibitors to coagulation factors and reduced bone density (RBD). RBD in bleeding disorders is becoming an escalating burden worldwide due to an increased in life expectancy and also in the ageing population with bleeding disorders. Here, we review published papers on bone mineral density of individuals with haemophilia A & B, a rare bleeding disorder entitled combined factor V and VIII deficiency and other rare bleeding disorders. It seems in individuals with bleeding disorders preventive measures including early detection, treatment and adequate physical activity, encouragement to be active in sports and finally anti-osteoporotic therapy must be done.
“…Reduced bone density in individuals with haemophilia is multi-factorial and if occurs during childhood phase, it is a major risk factor for acquisition osteopenia or osteoporosis in later of life [22]. RBD in severe haemophilia A is a concept which has been supported by several authors [3-5, 7, 11-18].…”
Section: Pathogenesis Of Reduced Bone Densitymentioning
In recent decays, quality of life and life expectancy of individuals with severe haemophilia A and B have been improved by better quality in treatment and more availability of blood coagulation products. Recently, new concerns have emerged in current groups such as auto-antibody inhibitors to coagulation factors and reduced bone density (RBD). RBD in bleeding disorders is becoming an escalating burden worldwide due to an increased in life expectancy and also in the ageing population with bleeding disorders. Here, we review published papers on bone mineral density of individuals with haemophilia A & B, a rare bleeding disorder entitled combined factor V and VIII deficiency and other rare bleeding disorders. It seems in individuals with bleeding disorders preventive measures including early detection, treatment and adequate physical activity, encouragement to be active in sports and finally anti-osteoporotic therapy must be done.
“…This deficiency results in recurrent bleeds into joints S. Phadke (&) Department of Medical Genetics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India e-mail: shubha@sgpgi.ac.in and muscles, leading to hemophilic arthropathy and contractures [1].…”
Approximately 14,000 people with hemophilia are registered at the Hemophilia Federation of India; however, hemophilia remains under-diagnosed and many cases are not registered. In June 2009, the Government of Uttar Pradesh made anti-hemophilic factors available at a few centers, including the Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow. Consequently, the level of hemophilia care has improved considerably in recent times. Amongst the many challenges facing people with hemophilia, the development of inhibitors, which neutralize clotting factors provided by replacement therapy, is the most feared one. Healthcare professionals who treat people with hemophilia should not only be knowledgeable about the condition and committed to bettering the management of hemophilia, but also take responsibility for the judicious allocation of resources for various aspects of managing hemophilia. This manuscript aims to raise awareness regarding the detection and management of inhibitors in hemophilia based on the experience of a tertiary care hemophilia treatment centre in Uttar Pradesh, India.
“…It is interesting that in most cases, fractures in PWH occur after minor trauma, such as a fall from a standing position, which are not common in the general population of these ages . Interestingly, this was also reported for femoral neck fractures . Stress fractures (i.e.…”
Section: Introductionmentioning
confidence: 95%
“…The average age at the time of sustaining a fracture is 28–30 years in PWH . Most common fracture sites in PWH are the lower limbs and, in particular, femur (mainly shaft and less frequently supracondylar and neck sites) and, to a lesser extent, tibia .…”
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