Sickle cell disease

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a mutation in the sixth amino acid of the β-globin gene (HBB). It is the most common serious genetic diseases in childhood, affecting approximately 1 in 2500 births and 100 000 individuals in the USA, in addition to 300 000 new cases globally each year. Central nervous system injury is the most debilitating frequent complication of SCD and includes stroke, silent cerebral infarct (SCI), and cognitive impairment. Among children with sickle cell anemia (HbSS), 11% had a stroke by age 18 years before the implementation of transcranial Doppler screening. SCI is identified in 27% of children with HbSS by their 5th birthday. Children who develop SCI have greater cognitive impairment compared with either children with HbSS without SCI or siblings without SCD. A recent study of adults demonstrated significant cognitive dysfunction, even in participants with apparently mild SCD.

Original languageEnglish (US)
Title of host publicationHandbook of Clinical Neurology
PublisherElsevier B.V.
Pages311-324
Number of pages14
Volume138
DOIs
StatePublished - 2016
Externally publishedYes

Publication series

NameHandbook of Clinical Neurology
Volume138
ISSN (Print)00729752
ISSN (Electronic)22124152

Fingerprint

Sickle Cell Anemia
Stroke
Nervous System Trauma
Hemoglobinopathies
Inborn Genetic Diseases
Globins
Siblings
Central Nervous System
Parturition
Amino Acids
Mutation
Genes
Cognitive Dysfunction

Keywords

  • cognitive impairment
  • hemorrhagic stroke
  • ischemic stroke
  • sickle cell anemia
  • sickle cell disease
  • silent cerebral infarct

ASJC Scopus subject areas

  • Medicine(all)
  • Neurology
  • Clinical Neurology

Cite this

Strouse, J. (2016). Sickle cell disease. In Handbook of Clinical Neurology (Vol. 138, pp. 311-324). (Handbook of Clinical Neurology; Vol. 138). Elsevier B.V.. https://doi.org/10.1016/B978-0-12-802973-2.00018-5

Sickle cell disease. / Strouse, John.

Handbook of Clinical Neurology. Vol. 138 Elsevier B.V., 2016. p. 311-324 (Handbook of Clinical Neurology; Vol. 138).

Research output: Chapter in Book/Report/Conference proceedingChapter

Strouse, J 2016, Sickle cell disease. in Handbook of Clinical Neurology. vol. 138, Handbook of Clinical Neurology, vol. 138, Elsevier B.V., pp. 311-324. https://doi.org/10.1016/B978-0-12-802973-2.00018-5
Strouse J. Sickle cell disease. In Handbook of Clinical Neurology. Vol. 138. Elsevier B.V. 2016. p. 311-324. (Handbook of Clinical Neurology). https://doi.org/10.1016/B978-0-12-802973-2.00018-5
Strouse, John. / Sickle cell disease. Handbook of Clinical Neurology. Vol. 138 Elsevier B.V., 2016. pp. 311-324 (Handbook of Clinical Neurology).
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