الفهرس 
AcknowledgementOnly 14 pages are availabe for public view
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Abstract
Hemophilia is an X-linked congenital bleeding disorder caused by deficiency of coagulation factor VIII (in hemophilia A) or factor IX (in hemophilia B). The deficiency is the result of mutations of the respective clotting factor genes.
Haemophilia is rare, with only about 1 instance in every 10,000 births (or 1 in 5,000 male births) for haemophilia A and 1 in 50,000 births for haemophilia B. According to the World Federation of Hemophilia, 400 000 people worldwide & 5,307 people in Egypt are suffering from hemophilia. Hemophilia A is more common than hemophilia B, representing 80-85 % of the total hemophilia population.
It is subjective representation of health, including not only physical, mental and social, but also emotional and everyday life dimensions in terms of wellbeing. Several definitions of QoL have been provided, the definition of the WHO viewing QoL as ’individuals perceptions of their position in life in the context of culture and value systems in which they live and in relation to their goals, expectation standards and concerns’
The aim of this study was to describe the health status, health care received, and their impact on the quality of life in patients with hemophilia.
Our prospective study included hemophilic patients of different age groups, of both types (Hemophilia A & B) and previously diagnosed in Asyut University Hospital.
The results of our present study can be summarized as follows:
• The present study showed that, the vast majority of the studied hemophilia cases were hemophilia A and 16% were hemophilia B.
• In this study, by comparing different parameters of SF-36 questionnaire between Hemophilia studied cases and their matched healthy controls we found that; for all SF-36 items (general health, limitation of activities, physical health problems, emotional health, social activities, pain, and energy and emotions), hemophilia cases suffered from significantly lower quality of life as compared to control group (<0.001).
• Among studied hemophilia cases; we comparing different parameters of SF36 questionnaire according to their age and we found that; younger aged patients (≤ 25 years) suffered from lower quality of life regarding limitation of activities (P=0.021), physical health problems (P=0.015), emotional health (P=0.007), energy and emotions (P=0.024), and their general health (P=0.016).
• In this study, among studied hemophilia cases; we comparing different parameters of SF36 questionnaire according to their occupation and we found that; unemployed patients suffered from lower quality of life regarding to their general heath (P=0.001), limitation of activities (P<0.00) ), and physical health problems (P=0.041).
• We comparing different parameters of SF-36 questionnaire between hemophilia patients type A versus hemophilia patients type B and we found that; hemophilia patients type A suffered from lower quality of life regarding all SF-36 items (general health, limitation of activities, physical health problems, emotional health, social activities, pain, and energy and emotions), (P<0.05).
• In this study, increasing the disease severity was associated with poorer quality of life among our studied cases as we found that; patients suffered from severer disease were recorded lower score for all SF-36 items compared to patients with mild or moderate disease severity status (general health, limitation of activities, physical health problems, emotional health, social activities, pain, and energy and emotions), (P<0.05).
• The current study revealed that, by using Friedman test to identifying the most worse problem the affecting the QOL among our studied cases we observed that; lower general health was the most problem that recorded by our studied cases (P<0.001).