Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Hydroxycarbamide treatment and brain MRI/MRA findings in children with sickle cell anaemia
source: British Journal of Haematology
year: 2016
authors: Nottage KA, Ware RE, Aygun B, Smeltzer M, Kang G, Moen J, Wang WC, Hankins JS, Helton KJ
summary/abstract:Silent cerebral infarction (SCI) is the most common neurological abnormality among children with sickle cell anaemia (SCA). The effect of hydroxycarbamide (also termed hydroxyurea) on the development and progression of SCI is unclear. We evaluated brain magnetic resonance imaging/angiography (MRI/MRA) in children with SCA receiving long-term hydroxycarbamide therapy. Fifty participants (median 9·4 years, range 1·1-17·3) enrolled in the Hydroxyurea Study of Long-Term Effects (HUSTLE; NCT00305175) underwent brain MRI/MRA and laboratory evaluations before hydroxycarbamide initiation and after 3 and 6 years of treatment to maximum tolerated dose. SCI and vascular stenosis were evaluated. At baseline, 3 and 6 years, SCI were present in 19/50 (38%), 20/49 (41%), and 7/17 (41%), respectively. At 3 years, one child developed a SCI lesion, and another progressed (single lesion to multiple). Lower haemoglobin (Hb) (80 g/l vs. 86 g/l, P = 0·049), fetal Hb (5·0% vs. 10·4%, P < 0·001) and oxygen saturation (97% vs. 98%, P = 0·027) before hydroxycarbamide initiation were associated with SCI. No patients had vascular stenosis identified on MRA, transient ischaemic attack or stroke. Our data indicate that children receiving hydroxycarbamide over a 3- to 6-year period have a low rate of new or worsening cerebrovascular disease. Further studies are needed to confirm that hydroxycarbamide can prevent the onset and progression of SCI.
organization: St. Jude Children's Research Hospital, Memphis, USA; Cincinnati Children's Hospital Medical Center; Steven and Alexandra Cohen Children's Medical Center of New York; University of MemphisDOI: 10.1111/bjh.14235
read more full text
+myBinderRelated Content
-
Laser therapy for retinopathy in sickle cell diseaseBACKGROUND: Sickle cell disease include...
-
Only 21% of Kids with ADHD and SCD Are Treated for Attention DeficitA study of children with sickle cell dis...
-
Presence of pain on three or more days of the week is associated with worse patient reported outcomes in adults with...While acute episodic pain is the hallmar...
-
Partnership to Ensure Supply of LentiGlobin, Potential Gene TherapyBluebird Bio extended a partnership with...
-
FDA Approves Novel Treatment to Target Abnormality in Sickle Cell DiseaseToday, the U.S. Food and Drug Administra...
-
MaxCyte, Inc. to Present Positive Preclinical Data for Sickle Cell DiseaseMaxCyte, a US-based global company dedic...
-
Solution to 50-year-old mystery could lead to gene therapy for common blood disordersIn a landmark study that could lead to 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.