Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
A systematic review of the literature for severity predictors in children with sickle cell anemia
source: Blood Cells, Molecules, and Diseases
year: 2017
authors: Meier ER, Fasano RM, Levett PR
summary/abstract:All patients with HbSS (SCA) share the same genetic mutation but the clinical phenotype is variable and difficult to predict early in life. A reliable severity predictor would be invaluable toward directing therapeutic decisions in those patients at highest risk of SCA complications. A search of PubMed, Cochrane Clinical Trials Register, and Scopus was performed to determine which SCA severity predictors have been validated in pediatric patients. The full text of 94 of the 590 references identified was reviewed based on the title/abstract. Fifty-four articles were included in the analysis. Alpha globin gene number was the most commonly studied severity predictor, followed by fetal hemoglobin (HbF) and reticulocyte count. Alpha thalassemia trait was protective against overt stroke and abnormal transcranial Doppler (TCD) in all but one study, but not frequency of painful crisis or silent cerebral infarct. Two thirds of the HbF studies reported beneficial effects with increasing HbF levels; however, increased HbF levels were not associated with lower hospitalization or stroke rates in others. The ability to predict SCA complications was mixed for all variables, except TCD and absolute reticulocyte count. More reliable predictors are urgently needed to guide therapeutic decisions in children with SCA.
organization: Indiana Hemophilia and Thrombosis Center; Emory University School of Medicine; The George Washington University School of Medicine and Health Sciences, Washington, DCDOI: 10.1016/j.bcmd.2017.01.014
read more full text
+myBinderRelated Content
-
Perspectives and practices of athletic trainers and team physicians implementing the 2010 NCAA sickle cell trait scr...Sickle cell trait (SCT) is usually benig...
-
Identifying factors underlying the decision for sickle cell carrier screening among African Americans within middle ...Guidelines recommend that African Americ...
-
Researchers Identify Genetic Predictors of Sickle Cell Anemia-Related ComplicationsPatients with sickle cell anemia who do ...
-
MARAC Statement: Crizanlizumab (Adakveo)July 7, 2023 - SCDAA’s Medical and Res...
-
Isaac Odame, MB ChB, MRCP, FRCPath, FRCPCH, FRCPCDr. Isaac Odame is a staff physician and...
-
National Sickle Cell Disease Poll of African Americans Dispels Long-Held ViewsPfizer Inc. (NYSE:PFE), the National New...
-
IASCNAPA Sickle Cell Disease Conference: Treating the Whole PersonDate: April 14-15, 2021 Place: Online/V...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.
Powered by
Support for this site is provided by
This platform is made possible through a partnership with the Sickle Cell Disease Association of America, Inc. (SCDAA) and its member organizations. SCDAA's mission is to advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure.