Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease
source: British Journal of Haematology
year: 2016
authors: Badawy SM, Liem RI, Rigsby CK, Labotka RJ, DeFreitas RA, Thompson AA
summary/abstract:Transfusional iron overload represents a substantial challenge in the management of patients with sickle cell disease (SCD) who receive chronic or episodic red blood cell transfusions. Iron-induced cardiomyopathy is a leading cause of death in other chronically transfused populations but rarely seen in SCD. Study objectives were to: (i) examine the extent of myocardial and hepatic siderosis using magnetic resonance imaging (MRI) in chronically transfused SCD patients, and (ii) evaluate the relationship between long-term (over the 5 years prior to enrolment) mean serum ferritin (MSF), spot-ferritin values and liver iron content (LIC) measured using MRI and liver biopsy. Thirty-two SCD patients (median age 15 years) with transfusional iron overload were recruited from two U.S. institutions. Long-term MSF and spot-ferritin values significantly correlated with LIC by MRI-R2* (r = 0·77, P < 0·001; r = 0·82, P < 0·001, respectively). LIC by MRI-R2* had strong positive correlation with LIC by liver biopsy (r = 0·98, P < 0·001) but modest inverse correlation with cardiac MRI-T2* (r = -0·41, P = 0·02). Moderate to severe transfusional iron overload in SCD was not associated with aberrations in other measures of cardiac function based on echocardiogram or serum biomarkers. Our results suggest that SCD patients receiving chronic transfusions may not demonstrate significant cardiac iron loading irrespective of ferritin trends, LIC and erythropoiesis suppression.
organization: Northwestern University, Chicago; Ann & Robert H. Lurie Children's Hospital of Chicago; University of Illinois School of MedicineDOI: 10.1111/bjh.14277
read more full text
+myBinderRelated Content
-
Sickle cell patients in UK survey, especially those 16 to 20, voice problems with care and pain relief givenIn a survey, patients across the...
-
EXpanding Treatment for Existing Neurological Disease (EXTEND): An Open-Label Phase II Clinical Trial of Hydroxyurea...Background: Cerebral vasculopathy in s...
-
3 Gene Editing Approaches for Sickle Cell DiseaseSickle cell disease (SCD) is a perfect c...
-
Results from Part A of the Hemoglobin Oxygen Affinity Modulation to Inhibit HbS Polymerization (HOPE) Trial (GBT440-...Sickle cell disease (SCD) is a genetic d...
-
FDA grants CRISPR gene therapy fast track designation for sickle cell diseaseThe FDA granted fast track designation t...
-
What Should Teens Ask Their Doctors About Hydroxyureahttps://www.youtube.com/watch?v=VZn15uXQ...
-
Global Blood Therapeutics Announces Completion of Safety Review by Independent Data and Safety Monitoring Board (DSM...Global Blood Therapeutics, Inc. (GBT) (N...
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.