How I use hydroxyurea to treat young patients with sickle cell anemia

RE Ware - Blood, The Journal of the American Society of …, 2010 - ashpublications.org
Hydroxyurea has many characteristics of an ideal drug for sickle cell anemia (SCA) and
provides therapeutic benefit through multiple mechanisms of action. Over the past 25 years …

Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management

SK Ballas, MR Kesen, MF Goldberg… - The Scientific World …, 2012 - Wiley Online Library
The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG→ GTG) in
exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position …

Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia

EP Vichinsky, LD Neumayr, JI Gold, MW Weiner… - Jama, 2010 - jamanetwork.com
Context Sickle cell anemia (SCA) is a chronic illness causing progressive deterioration in
quality of life. Brain dysfunction may be the most important and least studied problem …

Sickle cell disease

MJ Bonner, ES Puffer, VW Willard - Textbook of pediatric …, 2010 - books.google.com
Sickle cell disease (SCD) is a group of inherited autosomal recessive disorders, including
sickle cell anemia, sickle beta-thalassemia, and other hemoglobinopathies, which are …

Sickle cell disease: clinical presentation and management of a global health challenge

ME Houwing, PJ De Pagter, EJ Van Beers, BJ Biemond… - Blood reviews, 2019 - Elsevier
Sickle cell disease is an autosomal recessive, multisystem disorder, characterised by
chronic haemolytic anaemia, painful episodes of vaso-occlusion, progressive organ failure …

Hydroxyurea treatment and neurocognitive functioning in sickle cell disease from school age to young adulthood

AM Heitzer, J Longoria, V Okhomina… - British Journal of …, 2021 - Wiley Online Library
Neurocognitive impairment is common in sickle cell disease (SCD) and is associated with
significant functional limitations. In a cross‐sectional analysis, we examined the association …

Update on the use of hydroxyurea therapy in sickle cell disease

TE Wong, AM Brandow, W Lim… - Blood, The Journal of …, 2014 - ashpublications.org
Case 2: A 5-year-old girl with HbSS is being seen for a routine follow-up visit. She has never
had acute chest syndrome or other sickle cell–related complications. She currently has …

Intelligence quotient in paediatric sickle cell disease: a systematic review and meta‐analysis

JM Kawadler, JD Clayden, CA Clark… - … Medicine & Child …, 2016 - Wiley Online Library
Aim Sickle cell disease (SCD) is the commonest cause of childhood stroke worldwide.
Magnetic resonance imaging (MRI) is routinely used to detect additional silent cerebral …

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

AM Hood, AA King, ME Fields, AL Ford… - Pediatric blood & …, 2019 - Wiley Online Library
Individuals with sickle cell disease (SCD) experience cognitive deficits; however, it remains
unclear whether medical treatments for SCD improve cognition. Given that executive …

Chronic organ failure in adult sickle cell disease

E Vichinsky - Hematology 2014, the American Society of …, 2017 - ashpublications.org
Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan
failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes …