Related Experiment Video

Updated: Jul 16, 2026

Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases
11:08

Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases

Published on: June 22, 2012

The association of oral hydroxyurea therapy with improved cognitive functioning in sickle cell disease

Eve Puffer1, Jeffrey Schatz, Carla W Roberts

  • 1Department of Psychology, University of South Carolina, SC 29208, USA. evepuffer@gmail.com

Child Neuropsychology : a Journal on Normal and Abnormal Development in Childhood and Adolescence
|March 17, 2007
PubMed

Insights

Oral hydroxyurea therapy may improve cognitive function in children with sickle cell disease (SCD). Children on hydroxyurea showed higher scores in verbal comprehension and reasoning, suggesting potential brain benefits.

Area of Science:

  • Pediatric Hematology
  • Neuroscience
  • Clinical Therapeutics

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells.
  • Cognitive impairments are a recognized complication in children with SCD.
  • Hydroxyurea is a medication used to manage SCD symptoms.

Purpose of the Study:

  • To investigate the potential cognitive benefits of oral hydroxyurea therapy in pediatric patients with SCD.
  • To compare cognitive performance between children with SCD receiving hydroxyurea and those not on the medication.

Main Methods:

  • A comparative study design was employed.
  • Cognitive abilities were assessed in 15 children with SCD on hydroxyurea and 50 children with SCD not on the drug.
  • Demographics and hematocrit levels were controlled for in the analysis.

Main Results:

  • Children with SCD on hydroxyurea therapy demonstrated significantly higher scores on tests of verbal comprehension.
  • Fluid reasoning and general cognitive ability were also significantly improved in the hydroxyurea group.
  • These findings suggest a positive impact of hydroxyurea on cognitive functions.

Conclusions:

  • Preliminary evidence indicates that oral hydroxyurea therapy may confer cognitive benefits to children with SCD.
  • Potential mechanisms include improved cerebral blood/oxygen supply or reduced illness burden and fatigue.
  • Further research is warranted to confirm these cognitive advantages and elucidate underlying pathways.

More Related Videos

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry08:23

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

Published on: November 5, 2019

Related Concept Videos

Oral Hypoglycemic Agents: Sulfonylureas01:17

Oral Hypoglycemic Agents: Sulfonylureas

Sulfonylureas are oral hypoglycemic agents utilized in treating type 2 diabetes. They are characterized by their unique sulfonylurea chemical structure. The family of sulfonylureas is divided into generations. First-generation sulfonylureas, including tolbutamide (Orinase), chlorpropamide (Diabinese), and tolazamide (Tolinase), trigger insulin release from pancreatic β cells and enhance peripheral tissues' insulin sensitivity. The second-generation members, such as glipizide (Glucotrol),...
Alzheimer's Disease: Treatment01:22

Alzheimer's Disease: Treatment

Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
Cancer Therapies02:49

Cancer Therapies

Cancer therapies are various modes of treatment, such as surgery, radiation therapy, and chemotherapy that are administered to cancer patients.
However, cancer treatments can pose several challenges, as therapies used to kill cancer cells are generally also toxic to normal cells. Moreover, cancer cells mutate rapidly and can develop resistance to chemical agents or radiation therapy. Besides, all types of cancer cells may not respond to the same therapy. Some cancer cells respond to one...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
Hepatic Encephalopathy01:29

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...