PMID: 18205577Jan 22, 2008Paper

Management and clinical outcomes of transfusion-dependent thalassaemia major in an Australian tertiary referral clinic

The Medical Journal of Australia
Giselle L Kidson-GerberRobert Lindeman

Abstract

To evaluate the management, clinical outcomes and adherence to chelation therapy in adult transfusion-dependent patients with thalassaemia major. We reviewed all transfusion-dependent adults with thalassaemia major (n = 44) attending the Haematology Department at the Prince of Wales Hospital, Sydney, in 2005. Data were collected retrospectively (2000-2005) and prospectively (2005) for cross-sectional clinical audit from clinical reviews, patient questionnaires, pharmacy dispensing records and routine laboratory investigations. Iron overload and its complications; complications of transfusion; adherence to subcutaneous and oral chelation therapy (expressed as a percentage based on the ratio of the amount dispensed to the prescribed dose). The prevalence of diabetes mellitus was 18%; hypothyroidism, 16%; hypogonadism, 32%; cardiomyopathy, 9%; and osteopenia/osteoporosis, 83%. Serological evidence of exposure to hepatitis C and hepatitis B was present in 41% and 14% of patients, respectively, and 23% of patients had active hepatitis C infection. Predictors of complications included increasing number of years of transfusion, increasing age, coprescription of desferrioxamine and deferiprone, and poor adherence to desferrioxamine tre...Continue Reading

References

Jan 1, 1996·Acta Haematologica·V Gabutti, A Piga
Dec 11, 2002·JAMA : the Journal of the American Medical Association·Heather P McDonaldR Brian Haynes
Feb 28, 2004·Blood·Melody J CunninghamUNKNOWN Thalassemia Clinical Research Network
Jun 8, 2006·Journal of Cardiovascular Magnetic Resonance : Official Journal of the Society for Cardiovascular Magnetic Resonance·M A TannerD J Pennell

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