Sickle-cell disease

@article{Rees2010SicklecellD,
  title={Sickle-cell disease},
  author={David C. Rees and Thomas N. Williams and Mark T. Gladwin},
  journal={The Lancet},
  year={2010},
  volume={376},
  pages={2018-2031}
}
Sickle-cell disease is one of the most common severe monogenic disorders in the world. Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to the pathophysiology of this disease, although the importance of chronic anaemia, haemolysis, and vasculopathy has been established. Clinical management is basic and few treatments have a robust evidence base. One of the main problems of sickle-cell disease in children is the development of cerebrovascular disease and… Expand
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TLDR
The effectiveness of any intervention in preventing or reducing kidney complications or chronic kidney disease in people with SCD, including red blood cell transfusions, hydroxyurea and angiotensin-converting enzyme inhibitor (ACEI), either alone or in combination with each other is assessed. Expand
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TLDR
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TLDR
Hematopoietic stem cell transplantation is the only cure available today, but is not feasible for the vast majority of people suffering from SCD. Expand
Vasculopathy and pulmonary hypertension in sickle cell disease.
TLDR
An overview of the pathobiology of hemolysis-mediated endothelial dysfunction and eDAMPs is provided and a summary of the present understanding of diagnosis and management of pulmonary hypertension in sickle cell disease is summarized, including a review of recent American Thoracic Society (ATS) consensus guidelines for risk stratification and management. Expand
Pulmonary complications in adult patients with sickle cell disease
TLDR
The present review comprises the following pulmonary complications of SCD: i) acute chest syndrome, ii) venous thromboembolus, iii) asthma, iv) respiratory dysfunction, v) respiratory disorders during sleeping, vi) pulmonary hypertension. Expand
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TLDR
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TLDR
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