[Pathophysiology and treatment of sickle-cell disease].

  title={[Pathophysiology and treatment of sickle-cell disease].},
  author={Eduard J van Beers and Marjolein Peters and Bart J. Biemond},
  journal={Nederlands tijdschrift voor geneeskunde},
  volume={149 21},
Sickle-cell disease is a hereditary haemoglobinopathy caused by a mutation in the beta-globin gene. The disease is characterised by recurrent vaso-occlusive crises resulting in severe organ damage and a sharply reduced life expectancy. The formation of haemoglobin-S polymers in hypoxic conditions plays a pivotal role in sickle-cell disease and produces the characteristic phenotype of sickle-shaped erythrocytes that promote vasoocclusion. Endothelial cell activation, enhanced erythrocyte and… CONTINUE READING
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