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
-
Altered Oxygenation, Vascular and Ischemic Pain Responses in Adults with Sickle Cell AnemiaThe clinical hallmark of sickle cell ane...
-
Roland B. Scott Memorial Symposium: I Want Somebody to LoveRoland B. Scott Symposium on Sickle Cell...
-
Living With Unpredictable Pain: A Sickle Cell Disease StoryGrowing up, Vismel Marquez wanted to joi...
-
The Life, Death, and Dream of a Research Diversity Crusader“We want to have a nation in which the...
-
Preventing cognitive decline in sickle cell disease: A good night’s sleepAmong children with sickle cell disease ...
-
Sickle cell warrior documentaryhttps://www.youtube.com/watch?v=ZxC4vtN2...
-
CTX001 Earns FDA’s Fast Track Status for Treating Sickle Cell DiseaseThe U.S. Food and Drug Administration (F...
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.