BACKGROUND: Inherited bleeding disorders registries can be useful to improve health care of these rare disorders and document their natural history. MATERIAL AND METHODS: We analysed the epidemiological, diagnostic and therapeutic aspects of patients managed at an Italian Regional Haemophilia Centre, based in Pescara (in the Region of Abruzzo). RESULTS: This Regional Haemophilia Centre currently follows 376 patients: 248 with rare clotting factor defects, 33 with von Willebrand's disease, 75 with haemophilia A and 20 with haemophilia B. Three patients with severe haemophilia A have developed inhibitors. Among all the haemophiliacs, the prevalence of hepatitis C virus infection is 21% while the prevalence of human immunodeficiency virus infection is 5.3%. Among the whole haemophilic population referring to the Pescara Haemophilia Centre, 87.4% are treated with recombinant factors while 12 patients with severe haemophilia are receiving primary prophylaxis. CONCLUSION: In brief, an analysis of the epidemiological, clinical and therapeutic data collected at the Regional Haemophilia Centre of Pescara is a useful tool for monitoring and continuously improving the quality of care of patients with inherited bleeding disorders.
BACKGROUND:Inherited bleeding disorders registries can be useful to improve health care of these rare disorders and document their natural history. MATERIAL AND METHODS: We analysed the epidemiological, diagnostic and therapeutic aspects of patients managed at an Italian Regional Haemophilia Centre, based in Pescara (in the Region of Abruzzo). RESULTS: This Regional Haemophilia Centre currently follows 376 patients: 248 with rare clotting factor defects, 33 with von Willebrand's disease, 75 with haemophilia A and 20 with haemophilia B. Three patients with severe haemophilia A have developed inhibitors. Among all the haemophiliacs, the prevalence of hepatitis C virus infection is 21% while the prevalence of human immunodeficiency virus infection is 5.3%. Among the whole haemophilic population referring to the Pescara Haemophilia Centre, 87.4% are treated with recombinant factors while 12 patients with severe haemophilia are receiving primary prophylaxis. CONCLUSION: In brief, an analysis of the epidemiological, clinical and therapeutic data collected at the Regional Haemophilia Centre of Pescara is a useful tool for monitoring and continuously improving the quality of care of patients with inherited bleeding disorders.
Authors: V Ivaskevicius; R Jurgutis; S Rost; A Müller; C Schmitt; K Wulff; F H Herrmann; C R Müller; R Schwaab; J Oldenburg Journal: Br J Haematol Date: 2001-03 Impact factor: 6.998
Authors: A Tagliaferri; G F Rivolta; C Biasoli; L Valdré; G Rodorigo; M D'Incá; S Moratelli; P Albertini; D Vincenzi; M C Arbasi; M Marietta; C Pattacini Journal: Haemophilia Date: 2007-12-10 Impact factor: 4.287