Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance
2008

Improved Survival of Thalassaemia Major in the UK

Sample size: 850 publication Evidence: high

Author Information

Author(s): Bernadette Modell, Maren Khan, Matthew Darlison, Mark A Westwood, David Ingram, Dudley J Pennell

Primary Institution: UCL Centre for Health Informatics and Multiprofessional Education (CHIME)

Hypothesis

The study aimed to assess the impact of T2* cardiovascular magnetic resonance on survival rates in patients with thalassaemia major in the UK.

Conclusion

There has been a marked improvement in survival in thalassaemia major in the UK, primarily due to a reduction in deaths from cardiac iron overload.

Supporting Evidence

  • From 1980 to 1999, there were 12.7 deaths from all causes per 1,000 patient years.
  • In 2000–2003, the death rate from all causes fell significantly to 4.3 per 1,000 patient years.
  • The death rate from iron overload fell from 7.9 to 2.3 deaths per 1,000 patient years.

Takeaway

Kids with a blood condition called thalassaemia are living longer now because doctors can see and treat heart problems better.

Methodology

The study analyzed data from the UK Thalassaemia Register, including death certificates and clinical records of patients with thalassaemia major.

Limitations

The study is limited to data collected until the end of 2003, and the register has not been updated since then due to funding issues.

Participant Demographics

The study included 850 patients with thalassaemia major, primarily those who were transfusion-dependent before four years of age.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1186/1532-429X-10-42

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