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}
}
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616 Citations
Highly Influential Citations
33
Background Citations
179
Methods Citations
8
Results Citations
2

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616 Citations

Pulmonary hypertension in adolescents with sickle cell disease
TLDR
With vaso-occlusion being the major complication in sickle cell disease and affecting many tissues and organs like the lungs, pulmonary hypertension has become a major risk factor in patients, especially in children.
Sickle cell disease: Clinical presentation and management of a global health challenge.
Liver damage and sickle cell disease: genotype relationship
TLDR
Male sex, SCA genotype, lower HbF, frequent transfusions, increased GGT values, and abnormal liver ultrasound and stiffness were identified as potentially early markers of sickle hepatopathy.
Complement in sickle cell disease and targeted therapy: I know one thing, that I know nothing.
Interventions for chronic kidney disease in people with sickle cell disease.
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.
Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease.
TLDR
It is very uncertain whether long-term transfusions: reduce the risk of transient ischaemic attacks, Peto odds ratio, or increase quality of life across different outcomes according to GRADE methodology.
Sickle Cell Disease: A Brief Update.
Magnesium for treating sickle cell disease.
TLDR
The studies show that oral magnesium therapy may prevent sickle red blood cell dehydration and prevent recurrent painful episodes, and there is a need to access evidence for the impact of oral and intravenous magnesium effect on frequency of pain, length of hospital stay and quality of life.
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.
Current challenges in the management of patients with sickle cell disease – A report of the Italian experience
TLDR
The historical experience of the network of hemoglobinopathy centers and their approach to SCD in Italy is described, a country where hemoglobinopathies have a high prevalence and where SCD, associated with different genotypes including ß-thalassemia, is present in the native population.
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References

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Clinical research reviewed includes pulmonary hypertension, which is emerging as a particularly deadly scourge of adults with any haemolytic anaemia, and newer insights into older, more established complications and treatments, such as red cell transfusions, the need for and use of iron chelation and avascular necrosis.
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The pediatric experience of HCT for hemoglobinopathies is illustrated and how these results affect future therapeutic decisions in children who inherit these disorders is discussed.
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