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62 - Sickle Cell Disease

from Part III - Special Populations

Published online by Cambridge University Press:  15 December 2009

By
Suzanne Lippert
Edited by
Rachel L. Chin
Suzanne Lippert
Affiliation:
Alameda County Medical Center–Highland Campus, Oakland, CA
Rachel L. Chin
Affiliation:
University of California, San Francisco
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Summary

INTRODUCTION

Sickle cell hemoglobinopathy results from the substitution of a valine for glutamic acid as the sixth amino acid of the beta globin chain. Sickle cell disease (SCD) includes the most severe and most common form, homozygous sickle cell anemia (Hb SS), as well as the heterozygous condition of intermediate severity, S combined with hemoglobin C (Hb SC). Hemoglobin S is also seen in combination with beta thalassemia producing the more severe form, HbS-β0 thalassemia, and the less severe HbS-β+ thalassemia. The heterozygous (carrier) form in which hemoglobin S combines with normal hemoglobin A (Hb AS) is usually asymptomatic.

The sickling of erythrocytes that results from the deoxygenation-induced deformation of the hemoglobin S molecule causes erythrostasis, thrombosis, and ultimately infarction in the spleen of affected patients. SCD patients develop early functional asplenia, usually by 2–4 years of age, which leads to susceptibility to encapsulated organisms: Streptococcus pneumoniae, Haemophilus influenzae, Mycoplasma pneumoniae, Chlamydia pneumoniae. Prophylactic penicillin, given as 125 mg orally twice a day until 2–3 years of age and 250 mg twice daily until at least 5 years of age, is recommended as prevention against invasive pneumococcal disease.

EPIDEMIOLOGY

According to a World Health Organization report from 2005, “Sickle cell anemia affects millions throughout the world. In the United States, it affects around 72,000 people.

Type
Chapter
Information
Emergency Management of Infectious Diseases , pp. 409 - 414
Publisher: Cambridge University Press
Print publication year: 2008

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References

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  • Sickle Cell Disease
    • By Suzanne Lippert, Alameda County Medical Center–Highland Campus, Oakland, CA
  • Edited by Rachel L. Chin, University of California, San Francisco
  • Book: Emergency Management of Infectious Diseases
  • Online publication: 15 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547454.063
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Save book to Dropbox

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  • Sickle Cell Disease
    • By Suzanne Lippert, Alameda County Medical Center–Highland Campus, Oakland, CA
  • Edited by Rachel L. Chin, University of California, San Francisco
  • Book: Emergency Management of Infectious Diseases
  • Online publication: 15 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547454.063
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Sickle Cell Disease
    • By Suzanne Lippert, Alameda County Medical Center–Highland Campus, Oakland, CA
  • Edited by Rachel L. Chin, University of California, San Francisco
  • Book: Emergency Management of Infectious Diseases
  • Online publication: 15 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547454.063
Available formats
×