Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study

@article{Wierenga2001SurvivalEF,
  title={Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study},
  author={Klaas J Wierenga and Ian Hambleton and Norma A Lewis and Sickle Cell Unit},
  journal={The Lancet},
  year={2001},
  volume={357},
  pages={680-683}
}

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Population estimates of sickle cell disease in the U.S.

  • K. Hassell
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  • 2010
...

References

SHOWING 1-10 OF 31 REFERENCES

Improved survival in homozygous sickle cell disease: lessons from a cohort study

TLDR
Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.

Mortality in sickle cell disease. Life expectancy and risk factors for early death.

TLDR
Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death.

Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease.

TLDR
Comparison of this study's overall mortality with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies, and this improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.

Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease.

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of

Diagnosis at birth improves survival of children with sickle cell anemia.

TLDR
Studies reported from New York City, Los Angeles, New Haven, and Jamaica clearly demonstrate the calendar era change in survival that has occurred during the 1970s and 1980s, and the greatest improvement is found among those children who have sickle cell anemia.

Newborn screening for sickle cell disease: effect on mortality.

TLDR
Ten years of experience in newborn screening for sickle cell disease have been summarized and hemoglobinopathies are the most prevalent congenital disease.

The Development of Haematological Changes in Homozygous Sickle Cell Disease: a Cohort Study from Birth to 6 Years

TLDR
Children with SS disease were partially protected from iron deficiency in early childhood, perhaps by increased intestinal absorption of iron, and the associated increase in intracellular haemoglobin concentration might be disadvantageous during this high risk period.

Early deaths in Jamaican children with sickle cell disease.

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in

The haematology of homozygous sickle cell disease after the age of 40 years

TLDR
Age-related decreases in HbF, total haemoglobin and platelet counts and a falling reticulocyte count are consistent with a progressive bone marrow failure which is not explained by the commonly occurring renal impairment in older SS patients.