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}
}

Figures and Tables from this paper

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Current challenges in the management of patients with sickle cell disease – A report of the Italian experience
TLDR
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References

SHOWING 1-10 OF 151 REFERENCES
Advances in clinical research in sickle cell disease
TLDR
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.
Hematopoietic cell transplantation for thalassemia and sickle cell disease: past, present and future
TLDR
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.
Sickle cell disease in Africa: burden and research priorities
TLDR
This review details the epidemiological and clinical background of SCD, with an emphasis on Africa, before identifying the research priorities that will provide the necessary evidence base for improving the management of African patients.
A CASE OF SICKLE CELL ANEMIA WITH NECROPSY
Sickle cell anemia is a new disease, in the sense that it is just coming into general attention. It appears to be a familial disease or status peculiar to the negro race. Chief of its characteristics
Sickle cell disease.
  • P. Lane
  • Medicine, Biology
    Pediatric clinics of North America
  • 1996
Cerebrovascular disease associated with sickle cell pulmonary hypertension
TLDR
Six patients with obliterative central nervous system vasculopathy who also have pulmonary hypertension and high hemolytic rate are reported, suggesting that cerebrovascular disease and pulmonary hypertension in sickle cell disease share common mechanisms, in particular, reduced nitric oxide bioactivity associated with particularly high‐grade hemolysis.
Sickle cell disease and the kidney.
TLDR
Kidney transplantation offers a major advantage to survival, and should be coupled with efforts toward prevention of recurrent disease, especially in patients with a history of sickle cell disease.
Two different forms of homozygous sickle cell disease occur in Saudi Arabia
TLDR
The disease in the Eastern province has many mild features consistent with the higher HbF levels and more frequent alpha thalassaemia but bone pathology (painful crises, avascular necrosis of the femoral head, osteomyelitis) remains common and the disease inThe West is more severe consistent withThe Benin haplotype suggesting an African origin.
Hemolysis-associated priapism in sickle cell disease.
TLDR
Case subjects with priapism had significantly lower levels of hemoglobin; higher levels of lactate dehydrogenase, bilirubin, and aspartate aminotransferase; and higher reticulocyte, white blood cell, and platelet counts, suggesting an association of priapist with increased hemolysis.
Cerebrovascular accidents in sickle cell disease: rates and risk factors.
TLDR
The highest rates of prevalence of CVA and incidence were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes, and the incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients.
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