Haemophilia in the paediatric department, KBC Split: prevention of bleeding

Abstract

Haemophilia is a genetic haemorrhagic disorder characterised by the absence of VIII or IX coagulation factors and is treated with supplemental factors VIII or IX. Haemophilia A is the most common and accounts for 70% of all inherited coagulopathies. At the Paediatric Department, KBC Split from January, 1st 2003 to February 15 2008 28 haemophiliacs were registered, 21 with haemophilia A (75%), and 7 with haemophilia B (25%). 8 (38%) patients had mild haemophilia A , 6 (29%) moderate, and 7 (33%) severe. Two children (29%) had moderate haemophilia B, and 5 (71%) severe. 12 (42%) patients received monthly prophylaxis, 6 with severe haemophilia A (67%), and 3 (33%) with severe haemophilia B. Children were treated in the outpatients’ clinic for 649 days, on average 6 days, most often because of an ankle, elbow, and knee injury or epistaxis. Children were hospitalized 17 times, 79% with haemophilia A, and 21% with haemophilia B, mostly because of head, frontal thoracic and joint injuries. Hospitalisation lasted on average 8 days. Inhibitors were not registered, while two children developed mild haemophilic arthropathy.