Sickle Cell Disease.
Publication/Presentation Date
1-1-2021
Abstract
Sickle cell disease is an umbrella term for a group of hemoglobinopathies characterized by the presence of 2 β-globin gene mutations or deletions, at least 1 of which is the point mutation that leads to the production of hemoglobin S. Sickle cell disease is associated with hemolytic anemia, significant chronic end-organ damage, and early death. In high-income countries, at least 95% of children with the disease survive into adulthood, resulting in a growing population of affected adults with significant comorbidities, complex medical issues, and not enough specialists to provide care. Hydroxyurea is the primary therapy, and recent advances in understanding disease pathophysiology have led to new therapies; progress in bone marrow transplant and gene editing has resulted in cure in some patients. The purpose of this review is to provide an overview of the diagnosis, common acute and chronic clinical manifestations, and best practices for management of sickle cell disease.
Volume
174
Issue
1
First Page
1
Last Page
1
ISSN
1539-3704
Published In/Presented At
Pecker, L. H., & Lanzkron, S. (2021). Sickle Cell Disease. Annals of internal medicine, 174(1), ITC1–ITC16. https://doi.org/10.7326/AITC202101190
Disciplines
Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods
PubMedID
33428443
Department(s)
Administration and Leadership
Document Type
Article