Unmasking hypertension in children and adolescents with sickle/beta-thalassemia

Sickle cell disease (SCD) is associated with increased risk of cardiovascular disease, although blood pressure (BP) levels have been reported to be lower in SCD patients compared to general population. Aims of the present study were to investigate the prevalence of BP phenotypes and levels of arterial stiffness in pediatric patients with SCD and to assess the differences with children at risk for hypertension. We included in the study 16 pediatric SCD (HbS/β-thalassemia, S/β-thal) patients and 16 consecutive children at risk for hypertension referred to our hypertension clinic that served as high-risk controls. All patients underwent ambulatory BP monitoring and measurement of carotid-femoral pulse wave velocity (PWV). S/β-thal patients had lower office systolic BP than the high-risk control group (115.43 ± 10.03 vs 123.37 ± 11.92, P = .05) but presented similar levels of day and night ambulatory BP. Office hypertension was found in 12.5% of the S/β-thal patients and in 43.8% of the high-risk controls (P = .06), while 18.8% of the S/β-thal patients and 25% of the high-risk controls presented hypertension by ambulatory BP levels (P = .21). All of the S/β-thal patients with ambulatory hypertension had night hypertension (one combined night and day hypertension) with office normotension (masked hypertension). S/β-thal patients and high-risk controls presented equal prevalence of masked hypertension (18.8%). Children and adolescents with S/β-thal present similar prevalence of BP phenotypes and levels of PWV with children at risk for hypertension. A significant number of children and adolescents with S/β-thal may have masked nighttime hypertension despite normal office BP levels.