Bokov Plamen, Koehl Berengere, Benzouid Chérine, Verlhac Suzanne, Missud Florence, Benkerrou Malika, Delclaux Christophe
Paris University, AP-HP, Robert Debré Hospital, Department of Physiology, INSERM NeuroDiderot, Paris, France.
Department of Hematology, Paris University, AP-HP, Robert Debré Hospital, Sickle Cell Disease Center, Hematology Unit, Paris, France.
Am J Hypertens. 2024 Apr 15;37(5):358-365. doi: 10.1093/ajh/hpae013.
An important prevalence (32%-45%) of masked hypertension has been reported in children with sickle cell disease (SCD). Stroke screening is well established using transcranial Doppler (TCD) ultrasound. The objectives of our proof-of-concept study in childhood SCD were to evaluate the prevalence of hypertension and its relationships with cerebral vasculopathy (TCD velocity) and to further evaluate in a subgroup of children the correlations of cardiovascular autonomic nervous system indices with TCD velocity.
Ambulatory blood pressure measurement (ABPM) and TCD velocity were obtained in children with SCD and in a restricted sample, cardiac sympathovagal balance using heart rate variability analyses, baroreflex sensitivity, and pulse wave velocity were measured.
In 41 children with SCD (median age 14.0 years, 19 girls, SS/Sβ + thalassemia/SC: 33/2/6), ABPM results showed masked hypertension in 2/41 (5%, 95% confidence interval, 0-11) children, consistent with the prevalence in the general pediatric population, elevated blood pressure (BP) in 4/41 (10%) children, and a lack of a normal nocturnal dip in 19/41 children (46%). Children with increased TCD velocity had lower nocturnal dipping of systolic BP. In the 10 participants with extensive cardiovascular assessment, increased TCD velocity was associated with parasympathetic withdrawal and baroreflex failure. Exaggerated orthostatic pressor response or orthostatic hypertension was observed in 7/10 children that was linked to parasympathetic withdrawal.
Autonomic nervous system dysfunction, namely loss of parasympathetic modulation, of SCD contributes to increase TCD velocity but is not associated with an increased prevalence of masked hypertension.
NCT04911049.
据报道，镰状细胞病（SCD）患儿中隐匿性高血压的患病率较高（32%-45%）。经颅多普勒（TCD）超声检查已广泛用于中风筛查。我们在儿童SCD中进行的概念验证研究的目的是评估高血压的患病率及其与脑血管病变（TCD速度）的关系，并在一组儿童亚组中进一步评估心血管自主神经系统指标与TCD速度的相关性。
对SCD患儿进行动态血压测量（ABPM）和TCD速度测量，并在一个受限样本中，使用心率变异性分析、压力反射敏感性和脉搏波速度测量心脏交感迷走神经平衡。
在41例SCD患儿（中位年龄14.0岁，19名女孩，SS/Sβ+地中海贫血/SC：33/2/6）中，ABPM结果显示2/41（5%，95%置信区间，0-11）例患儿存在隐匿性高血压，与普通儿科人群的患病率一致，4/41（10%）例患儿血压升高，19/41例患儿（46%）夜间血压缺乏正常下降。TCD速度增加的患儿夜间收缩压下降幅度较小。在10名进行广泛心血管评估的参与者中，TCD速度增加与副交感神经活动减弱和压力反射功能衰竭有关。7/10例患儿出现过度的直立性升压反应或直立性高血压，这与副交感神经活动减弱有关。
SCD的自主神经系统功能障碍，即副交感神经调节丧失，会导致TCD速度增加，但与隐匿性高血压患病率增加无关。
NCT04911049。
