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Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
08:23

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

Published on: November 5, 2019

Sickle-cell disease.

David C Rees1, Thomas N Williams, Mark T Gladwin

  • 1Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, King's College London, London, UK. david.rees@kcl.ac.uk

Lancet (London, England)
|December 7, 2010
PubMed
Summary
This summary is machine-generated.

Sickle-cell disease (SCD) causes severe health issues due to red blood cell sickling. Effective treatments are limited, especially in Africa, highlighting the need for targeted therapies.

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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

Area of Science:

  • Genetics and Hematology
  • Pediatric Neurology
  • Global Health

Background:

  • Sickle-cell disease (SCD) is a prevalent severe monogenic disorder globally.
  • Pathophysiology involves haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion.
  • Chronic anaemia, haemolysis, and vasculopathy are established contributors to SCD complications.

Purpose of the Study:

  • To review the pathophysiology and clinical management of sickle-cell disease.
  • To highlight the challenges in treating SCD, particularly cerebrovascular disease and cognitive impairment in children.
  • To discuss the impact of geographic location, especially Africa, on SCD severity and outcomes.

Main Methods:

  • Literature review of sickle-cell disease pathophysiology, clinical manifestations, and current management strategies.
  • Analysis of the role of specific complications like cerebrovascular disease and cognitive impairment.
  • Examination of factors influencing disease severity, including infectious diseases in African populations.

Main Results:

  • Haemoglobin polymerisation is central to SCD pathophysiology.
  • Cerebrovascular disease and cognitive impairment are significant concerns in pediatric SCD.
  • SCD follows a more severe clinical course in Africa, influenced by infectious diseases.

Conclusions:

  • Current clinical management for SCD is basic with limited evidence-based treatments.
  • Further research is essential to develop targeted therapies for SCD pathophysiology and complications.
  • Understanding and addressing the increased severity of SCD in Africa is critical for global health equity.