Haemophilia A management in Victorian, New South Wales and South Australian haemophilia centres
作者:
Henry Ekert,
Nicole L Ekert,
Alison M Street,
Kevin A Rickard,
V Jean McPherson,
Ian R G Toogood,
John V Lloyd,
期刊:
Medical Journal of Australia
(WILEY Available online 1995)
卷期:
Volume 162,
issue 11
页码: 569-571
ISSN:0025-729X
年代: 1995
DOI:10.5694/j.1326-5377.1995.tb138544.x
出版商: Wiley
数据来源: WILEY
摘要:
ObjectiveTo examine the management of haemophilia A in Australia and to compare it with international trends.MethodsSix haemophilia centres treating most patients in Victoria, New South Wales and South Australia were surveyed in 1993 by means of a written questionnaire followed by an “on site” interview.ResultsThe centres were treating 739 patients; 234 (32%) had severe haemophilia. Factor VIII inhibitors were present in 5.9% of all patients and in 19% of those with severe disease. Twenty‐three per cent were human immunodeficiency virus (HIV) antibody‐positive and 74% were hepatitis C virus (HCV) antibody‐positive. The main treatment was “on demand” therapy for acute bleeds (average use of factor VIII: 1350 IU/kg per year for children; and 780 IU/kg per year for adults). Prophylactic therapy was used in only 17 patients, with doses of 3000–4500 IU/kg per year. One million IU was used for three patients with high litre inhibitors who had “tolerising” therapy. While most developed countries have a factor VIII supply of 2–5 IU per capita, the total supplied to the States represented 1.46 IU per capita, while use at the centres represented 1.1 IU per capita.ConclusionBecause supply of factor VIII is limited, use was less than half that recommended internationally. Shortage of factor VIII has compromised prophylactic therapy and virtually prevented “tolerising” therapy.
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