The DOI matches its Crossref record; 32 of the 32 references it cites were found in a registry or an index.
Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance
Open accessAbstract
BACKGROUND: The UK Thalassaemia Register records births, deaths and selected clinical data of patients with thalassaemia who are resident in the UK. A study of survival and causes of death was undertaken which aimed to include the possible impact of T2* cardiovascular magnetic resonance (CMR). METHODS: The Register was updated to the end of 2003, copies of death certificates were obtained, and causes of death in beta thalassaemia major were extracted. In addition, patients who had T2* CMR assessment of cardiac iron load and/or received the oral iron chelator deferiprone were identified from clinical records. RESULTS: The main causes of death were anaemia (before 1980), infections, complications of bone marrow transplantation and cardiac disease due to iron overload. From 1980 to 1999 there were 12.7 deaths from all causes per 1,000 patient years. Forty per cent of patients born before 1980 had T2* cardiovascular magnetic resonance between 2000 and 2003, and 36% of these patients were prescribed deferiprone before end of 2003. In 2000-2003, the death rate from all causes fell significantly to 4.3 per 1,000 patient years (-62%, p < 0.05). This was mainly driven by the reduction in the rate of deaths from iron overload which fell from 7.9 to 2.3 deaths per 1,000 patient years (-71%, p < 0.05). CONCLUSION: Since 1999, there has been a marked improvement in survival in thalassaemia major in the UK, which has been mainly driven by a reduction in deaths due to cardiac iron overload. The most likely causes for this include the introduction of T2* CMR to identify myocardial siderosis and appropriate intensification of iron chelation treatment, alongside other improvements in clinical care.
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Cites
Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload
Anderson, L. (2001) · European Heart Journal · doi:10.1053/euhj.2001.2822 · Cited by 1,682
Maceira, A., Prasad, S., Khan, M., & Pennell, D. (2006) · Journal of Cardiovascular Magnetic Resonance · doi:10.1080/10976640600572889 · Cited by 847
Self-citationThe Biochemistry of Desferrioxamine and its Relation to Iron Metabolism
Keberle, H. (1964) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1965.tb54077.x · Cited by 612
SURVIVAL AND CAUSES OF DEATH IN THALASSAEMIA MAJOR
Zurlo, M., De Stefano, P., Borgna-Pignatti, C., Di Palma, A., Melevendi, C., Piga, A., Di Gregorio, F., Burattini, M., & Terzoli, S. (1989) · The Lancet · doi:10.1016/s0140-6736(89)90264-x · Cited by 611
Survival in β-thalassaemia major in the UK: data from the UK Thalassaemia Register
Modell, B., Khan, M., & Darlison, M. (2000) · The Lancet · doi:10.1016/s0140-6736(00)02357-6 · Cited by 583
Self-citationPennell, D. J. (2006) · Blood · doi:10.1182/blood-2005-07-2948 · Cited by 495
Self-citationTanner, M. A., Galanello, R., Dessi, C., Smith, G. C., Westwood, M. A., Agus, A., Roughton, M., Assomull, R., Nair, S. V., Walker, J. M., et al. (2007) · Circulation · doi:10.1161/circulationaha.106.648790 · Cited by 482
Self-citationAnderson, L. J., Wonke, B., Prescott, E., Holden, S., Malcolm Walker, J., & Pennell, D. J. (2002) · The Lancet · doi:10.1016/s0140-6736(02)09740-4 · Cited by 430
Self-citationAnderson, L. J., Westwood, M. A., Holden, S., Davis, B., Prescott, E., Wonke, B., Porter, J. B., Malcolm Walker, J., & Pennell, D. J. (2004) · British Journal of Haematology · doi:10.1111/j.1365-2141.2004.05202.x · Cited by 414
Self-citationWestwood, M., Anderson, L. J., Firmin, D. N., Gatehouse, P. D., Charrier, C. C., Wonke, B., & Pennell, D. J. (2003) · Journal of Magnetic Resonance Imaging · doi:10.1002/jmri.10332 · Cited by 379
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On T2* Magnetic Resonance and Cardiac Iron
Carpenter, J.-P., He, T., Kirk, P., Roughton, M., Anderson, L. J., de Noronha, S. V., Sheppard, M. N., Porter, J. B., Walker, J. M., Wood, J. C., et al. (2011) · Circulation · doi:10.1161/circulationaha.110.007641 · Cited by 485
Self-citationIron overload in thalassemia: different organs at different rates
Taher, A. T., & Saliba, A. N. (2017) · Hematology · doi:10.1182/asheducation-2017.1.265 · Cited by 355
SCMR Position Paper (2020) on clinical indications for cardiovascular magnetic resonance
Leiner, T., Bogaert, J., Friedrich, M. G., Mohiaddin, R., Muthurangu, V., Myerson, S., Powell, A. J., Raman, S. V., & Pennell, D. J. (2020) · Journal of Cardiovascular Magnetic Resonance · doi:10.1186/s12968-020-00682-4 · Cited by 328
Self-citationReview article: the iron overload syndromes
Siddique, A., & Kowdley, K. V. (2012) · Alimentary Pharmacology & Therapeutics · doi:10.1111/j.1365-2036.2012.05051.x · Cited by 162
Gene Therapy of the β-Hemoglobinopathies by Lentiviral Transfer of the β A(T87Q) - Globin Gene
Negre, O., Eggimann, A.-V., Beuzard, Y., Ribeil, J.-A., Bourget, P., Borwornpinyo, S., Hongeng, S., Hacein-Bey, S., Cavazzana, M., Leboulch, P., et al. (2016) · Human Gene Therapy · doi:10.1089/hum.2016.007 · Cited by 159
Metadata
- Authors
- Modell, B., Khan, M., Darlison, M., Westwood, M. A., Ingram, D., & Pennell, D. J.
- Venue
- Journal of Cardiovascular Magnetic Resonance
- Publisher
- Elsevier BV
- Published
- 2008-01
- Volume
- 10
- Issue
- 1
- Pages
- 42
- ISSN
- 1097-6647 (ISSN-L)
- OpenAlex
- W2069918539
- Cited by
- 600
- References
- 32
- FWCI
- 13.98
- Type
- journal article
- Language
- en
- Open access
- gold
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