Related Experiment Video

Updated: May 26, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

Prospects for primary stroke prevention in children with sickle cell anaemia

Lori C Jordan1, James F Casella, Michael R DeBaun

  • 1Department of Neurology, Division of Pediatric Neurology, Vanderbilt University School of Medicine, Nashville, TN 37232, USA.

British Journal of Haematology
|January 10, 2012
PubMed

Insights

This review examines current stroke prevention methods for children with sickle cell anaemia (SCA), focusing on transcranial Doppler (TCD) screening and blood transfusions. It also explores alternative strategies like immunizations and novel therapies to prevent neurological injury.

Area of Science:

  • Pediatric Hematology
  • Neurology
  • Public Health

Background:

  • Sickle cell anaemia (SCA) poses a significant risk for ischaemic strokes in children.
  • Current primary stroke prevention relies on transcranial Doppler (TCD) ultrasound screening.
  • Blood transfusion therapy is the standard intervention based on TCD findings from clinical trials.

Purpose of the Study:

  • To review the strengths and limitations of the current standard of care for primary stroke prevention in pediatric SCA.
  • To discuss theoretical alternative strategies for preventing neurological injury in SCA patients.
  • To highlight potential new approaches for stroke risk reduction in this population.

Main Methods:

  • Literature review of existing studies on stroke prevention in SCA.
  • Analysis of the current standard of care: TCD screening and blood transfusions.
  • Exploration of theoretical frameworks for alternative preventative strategies.

Main Results:

  • The current TCD screening and transfusion protocol has demonstrated efficacy but also presents limitations.
  • Alternative strategies, including immunizations and blood pressure management, show promise, especially in resource-limited settings.
  • Novel therapies like hydroxycarbamide and hematopoietic stem cell transplant offer potential for improved long-term prevention.

Conclusions:

  • The current standard of care for stroke prevention in pediatric SCA has limitations that warrant further investigation.
  • Alternative and adjunctive strategies are crucial for comprehensive neurological protection in children with SCA.
  • Future research should focus on optimizing existing methods and validating novel approaches to reduce stroke incidence.

Related Concept Videos

Hemorrhagic Stroke ll: Pathophysiology01:29

Hemorrhagic Stroke ll: Pathophysiology

A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...
Hemorrhagic Stroke l: Introduction01:17

Hemorrhagic Stroke l: Introduction

A hemorrhagic stroke is an acute neurological event that occurs when a weakened cerebral blood vessel ruptures, allowing blood to accumulate within or around the brain. The sudden release of blood forms a focal hematoma that increases intracranial pressure, displaces neural tissue, and can obstruct cerebrospinal fluid pathways. These effects may be compounded by intraventricular extension of the hemorrhage, cerebral edema, or compression of adjacent structures, all of which contribute to...
Ischemic Stroke l: Introduction01:15

Ischemic Stroke l: Introduction

Ischemic stroke is an acute cerebrovascular condition in which blood flow to a brain region is suddenly interrupted, leading to tissue infarction. Neurons depend on continuous oxygen and glucose supply, so even brief reductions in perfusion cause energy failure, ionic imbalance, and irreversible injury. Ischemic strokes are classified into thrombotic and embolic types based on their underlying mechanisms.Thrombotic MechanismsThrombotic stroke develops when a clot forms within a cerebral artery.
Ischemic Stroke ll: Pathophysiology01:15

Ischemic Stroke ll: Pathophysiology

An ischemic stroke occurs when a cerebral blood vessel becomes obstructed, most often by a thrombus or embolus, interrupting the delivery of oxygen and glucose to brain tissue. Because neurons rely on continuous aerobic metabolism, energy failure begins within minutes of reduced perfusion. The region receiving the least blood flow becomes the infarct core, an area of irreversible cellular death. Surrounding this core lies the penumbra, a zone of hypoperfused but still viable tissue that is...