Sickle Cell Disease
Authors: Martin H. Steinberg
Journal: Annals of Internal Medicine (2011)

In the Clinic6 September 2011Sickle Cell DiseaseDeborah Cotton, MD, MPH, Darren Taichman, MD, PhD, Sankey Williams, MD, and Martin H. Steinberg, MDDeborah Cotton, MD, MPH, Darren Taichman, MD, PhD, Sankey Williams, MD, and Martin H. Steinberg, MDAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-155-5-201109060-01003 SectionsSupplemental MaterialAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Sickle cell disease is a Mendelian genetic disorder caused by several common genotypes (see the Box: Common Genotypes of Sickle Cell Disease). Sickle cell trait is clinically benign and should not be considered a disease.Common Genotypes of Sickle Cell DiseaseHbA is the major normal adult hemoglobin.Sickle cell anemia: homozygosity for the HbS or sickle hemoglobin geneHbSC disease: compound heterozygosity for HbS and HbC genesHbS–β-thalassemia: compound heterozygosity for HbS and a β0- or β+-thalassemia geneHbSO Arabia: compound heterozygosity for HbS and HbO ArabiaHbSD Los Angeles (Punjab): compound heterozygosity for HbS and HbDHbSE: compound heterozygosity for HbS and HbEMany other ...References1. Halasa NB, Shankar SM, Talbot TR, et al. Incidence of invasive pneumococcal disease among individuals with sickle cell disease before and after the introduction of the pneumococcal conjugate vaccine. Clin Infect Dis. 2007;44:1428-33. [PMID: 17479937] Google Scholar2. Adams R, McKie V, Nichols F, et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med. 1992;326:605-10. 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