title: Minireview: Clinical severity in sickle cell disease: the challenges of definition and prognostication.
abstract: Sickle cell disease (SCD) is a monogenic, yet highly phenotypically variable disease with multisystem pathology. This manuscript provides an overview of many of the known determinants, modifiers, and correlates of disease severity in SCD. Despite this wealth of data, modeling the variable and multisystem pathology of SCD continues to be difficult. The current status of prediction of specific adverse outcomes and global disease severity in SCD is also reviewed, highlighting recent successes and ongoing challenges.
authors.affiliation: Division of Hematology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45220, USA charles.quinn@cchmc.org.
authors.forename: Charles T
authors.initials: CT
authors.lastname: Quinn
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D005319:Fetal Hemoglobin / Q000032:analysis; D006801:Humans; D011379:Prognosis; D012720:Severity of Illness Index; D055544:beta-Globins / Q000032:analysis
chemical_list: D055544:beta-Globins; D005319:Fetal Hemoglobin
keywords: Biomarkers; disease severity; models; outcomes; phenotype; prediction; prevention; prognosis; sickle cell anemia; sickle cell disease; survival