Non-invasive detection of endothelial dysfunction in sickle cell disease by Doppler ultrasonography

S D Zawar1, M A Vyawahare, M Nerkar

  • 1Department of Medicine, Indira Gandhi Medical College, Nagpur.

Insights

Endothelial function is impaired in sickle cell disease (SCD), particularly in sickle cell anemia (SS) compared to sickle cell trait (SA). This dysfunction correlates with disease severity in SS patients.

Area of Science:

  • Vascular Biology
  • Hematology
  • Cardiovascular Medicine

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
  • Endothelial dysfunction is a key factor in SCD pathogenesis, contributing to vaso-occlusion and organ damage.
  • Assessing endothelial function provides insights into disease severity and progression.

Purpose of the Study:

  • To evaluate endothelial function in patients with sickle cell disease (SCD).
  • To compare endothelial dysfunction between sickle cell anemia (SS) and sickle cell trait (SA) individuals.
  • To investigate the correlation between endothelial dysfunction and clinical indicators of SCD severity, such as symptom duration and vaso-occlusive crises frequency.

Main Methods:

  • A study involving 37 steady-state SCD patients (19 SS, 18 SA) and 33 matched controls.
  • Endothelial function assessed using flow-mediated dilation (FMD) of the brachial artery via vascular Doppler.
  • Standardized pneumatic tourniquet stress applied to the forearm for FMD measurement.

Main Results:

  • Significantly impaired FMD in both SS cases (6.22% ± 0.91%) and SA cases (12.56% ± 0.90%) compared to controls (P<0.05).
  • Endothelial function was more severely impaired in SS patients than in SA patients (P<0.05).
  • A decline in endothelial function correlated with longer symptom duration and higher annual vaso-occlusive crisis rates in SS cases.

Conclusions:

  • Endothelial dysfunction is a significant finding in sickle cell disease.
  • Sickle cell anemia (SS) demonstrates more pronounced endothelial impairment than sickle cell trait (SA).
  • Endothelial function serves as a potential biomarker for disease severity in SCD.
Abstract

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