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Ηaemophilia is a rare chronic genetic bleeding disorder which was life-threatening until 1950 when research and knowledge in the field of haemostasis took place. The aim of the review is the introduction of the haemophilia disorder and the basic principles of nursing care as well as an exploration of the recent advances in the field of haemophilia. Haemophilia A and B is a sex linked genetic disorder resulting in deficiency of factor VIII and IX coagulant activity respectively. It is transmitted by a gene on the X chromosome occurring predominantly in males. Main clinical features s of haemophilia include: (a) haemorrhage, muscle haematoma (b) prolonged life-threatening post-operative bleeding (c) intracranial bleeding. Bleeding episodes are treated with the administration of lyophilized factor concentrate. Complications of factor replacement therapy were the infection of blood borne viruses HIV/HCV. This tragic incident brought the resurgence of the fear of stigmatization and discrimination in the haemophiliac community with devastating effects on their psychological status. Nowadays the developments in therapeutic product technology and molecular biology through the viral attenuation of plasma derived factor concentrates and the introduction of recombinant concentrates have eliminated the possibility of viral infections. The complications of haemophilia necessitate coordinated inter-disciplinary care in special treatment Haemophilia Centres where the ultimate goal is the empowerment of the haemophiliac population and its integration into the social fabric.
|Volume 47, N 2
|Electra Loukopoulou , Dimitra Antoniadou