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Hydroxyurea therapy for sickle cell disease in Britain
- Source :
- BMJ. 316:1689-1690
- Publication Year :
- 1998
- Publisher :
- BMJ, 1998.
-
Abstract
- Sickle cell disease is the commonest inherited haemoglobinopathy in Britain and affects over 9000 people.1 Clinical severity varies considerably, but patients with the most severe disease have a life expectancy of just over 40 years. Conventional management of the disease is largely supportive, highlighting a pressing need for approaches that can alter the course of the disease. Trials in America have suggested that hydroxyurea can have a significant impact on the course of the disease, but in Britain it is proving virtually impossible to recruit patients into trials to confirm these results in a British population. The fact that the clinical severity of the sickle cell disease varies even within groups of patients with the same β globin genotype2 has led to the concept that the disease is a multigene disorder, with inheritance of α thalassemia and genes controlling the concentration of fetal haemoglobin, among others, modulating disease expression. …
- Subjects :
- Pediatrics
medicine.medical_specialty
education.field_of_study
business.industry
Thalassemia
Cell
Population
General Engineering
Severe disease
General Medicine
Disease
medicine.disease
medicine.anatomical_structure
medicine
Fetal haemoglobin
Life expectancy
General Earth and Planetary Sciences
Clinical severity
business
education
General Environmental Science
Subjects
Details
- ISSN :
- 14685833 and 09598138
- Volume :
- 316
- Database :
- OpenAIRE
- Journal :
- BMJ
- Accession number :
- edsair.doi...........73be3a642c9a20873f8c73f719de9781