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Prevention of heart disease by subcutaneous desferoxamine in patients with thalassaemia major
Author(s) -
Richardson M. E.,
Matthews R. N.,
Alison J. F.,
Menahem S.,
Mitvalsky J.,
Byrt E.,
Harper R. W.
Publication year - 1993
Publication title -
australian and new zealand journal of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0004-8291
DOI - 10.1111/j.1445-5994.1993.tb04722.x
Subject(s) - medicine , disease , intensive care medicine , pediatrics
Background : Cardiac siderosis from transfused iron remains the major cause of death in thalassaemia major, despite iron chelation therapy with desferrioxamine. Aims : Our aim was to determine the nature and extent of cardiac involvement and its relationship to desferrioxamine use in a group with thalassaemia major. Methods : We reviewed 76 patients with thalassaemia major and performed multiple logistic regression to analyse factors affecting cardiac involvement. Factors studied included: patient sex, age, haemoglobin, serum ferritin, total transfusions, liver iron, duration of desferrioxamine use, electrocardiograms, echocardiograms and compliance to desferrioxamine treatment. Results: Thirty‐seven patients developed heart disease. They were older ( p < 0.001), began desferrioxamine later ( p < 0.001), had more liver iron ( p = 0.014), higher serum ferritin levels ( p = 0.023) and received more blood (p = 0.018). Compared to those with optimal compliance the odds of developing heart disease were increased 10.7 times in fair compilers ( p <0.001) and 5.1 times in poor compilers ( p = 0.016). However, there was no significant difference between those with fair and poor compliance. After multivariate analysis only compliance ( p = 0.02) and age at desferrioxamine onset ( p = 0.004) remained significant. Compliance was inversely related to liver iron ( p < 0.001), serum ferritin ( p < 0.001) and age at desferrioxamine commencement ( p < 0.001). Conclusions : We conclude that late commencement of desferrioxamine and noncompliance are associated with greater iron loading and an increased riskof heart disease. (Aust NZ J Med 1993; 23: 656–661.)