Sickle Cell Disease Related Vasculopathies and Early Evaluation in a Pediatric Population

Area of Science:

• Pediatric Hematology
• Cardiovascular Medicine
• Nephrology

Background:

• Cardiovascular pathologies are common in sickle cell disease (SCD).
• A literature review compared SCD vasculopathies (SCDVs) to the general population.
• Pediatric SCDVs were investigated retrospectively.

Purpose of the Study:

• To compare the epidemiology of SCDVs with the general population.
• To investigate SCDVs in a pediatric cohort.
• To identify early indicators of SCDVs in children.

Main Methods:

• Studied SCDVs in relation to patient age, β-globin genotypes, and fetal hemoglobin (HbF).
• Analyzed urine microalbumin/creatinine ratios (UM/Cr), trans-cranial Doppler (TCD), and tricuspid regurgitant jet velocities (TRJV).
• Descriptively presented retinographies and overt vasculopathies.

Main Results:

• Age-dependent trends and surrogate markers suggest early SCDV origination.
• Higher TRJV and overt vasculopathy correlated with older age and lower HbF.
• Cerebral, cardiopulmonary, and renal vasculatures evolved independently in a subpopulation.

Conclusions:

• Overt SCDVs are less frequent in children but show early trends.
• Age-dependent markers indicate early onset of SCDVs in youth.
• Intense screening is justified to prevent SCDV progression with disease-modifying treatments.