Ranabothu Saritha, Hafeman Michael, Manwani Deepa, Reidy Kimberly, Morrone Kerry, Lorenzo Josemiguel, Tria Barbara, Kaskel Frederick, Mahgerefteh Joseph
Pediatrics, University of Arkansas for Medical Sciences, Little Rock, USA.
Pediatrics, Albert Einstein College of Medicine, Bronx, USA.
Cureus. 2020 Nov 25;12(11):e11707. doi: 10.7759/cureus.11707.
Background Sickle cell disease (SCD), a chronic hemolytic disorder, results in cumulative end-organ damage affecting major organs such as the cardiovascular, renal, and central nervous systems. Effects of modifiable risk factors, such as blood pressure (BP), on the development of end-organ complications in SCD have not been well studied, particularly among the pediatric population. Relative hypertension in patients with SCD increases their risks of stroke, cardiovascular complications, and death. The primary hypothesis of this study was that abnormal BP patterns are common among patients with SCD and they impact end-organ complications. Methods Patients with SCD (HbSS, HbSβ0) were enrolled from the Children's Hospital at Montefiore (N = 100). For each patient, demographic data were collected, biochemical variables in urine and blood samples were analyzed, BP was determined with ambulatory blood pressure monitoring (ABPM), and an echocardiogram was performed. The prevalence of abnormalities in BP parameters was defined, and their relationships with measures of SCD severity and end-organ damage were assessed. Results Sufficient ABPM data were available for 67 patients. Enrolled children were 13 ± 4 years (40% were males). Assessment of diurnal variation demonstrated that 81% of patients had abnormal systolic nocturnal dipping and 61% had abnormal diastolic nocturnal dipping. Abnormalities in the diurnal pattern were associated with reticulocytosis and hyperfiltration. Microalbuminuria was present in 19% (n = 13) of patients, of which 77% (n = 10) were females (p = 0.014). Diastolic load and abnormal nocturnal dipping were associated with hyperfiltration but not with microalbuminuria. Conclusions BP abnormalities detected with ABPM in SCD patients are prevalent and perhaps are a risk factor for end-organ complications. Further studies are required to identify the mechanisms underlying these relationships and their longitudinal changes.
背景 镰状细胞病（SCD）是一种慢性溶血性疾病，会导致累积性终末器官损伤，影响心血管、肾脏和中枢神经系统等主要器官。可改变的危险因素，如血压（BP），对SCD患者终末器官并发症发生发展的影响尚未得到充分研究，尤其是在儿科人群中。SCD患者的相对高血压会增加其患中风、心血管并发症和死亡的风险。本研究的主要假设是，异常血压模式在SCD患者中很常见，并且会影响终末器官并发症。方法 从蒙特菲奥里儿童医院招募了SCD（HbSS、HbSβ0）患者（N = 100）。收集每位患者的人口统计学数据，分析尿液和血液样本中的生化变量，通过动态血压监测（ABPM）测定血压，并进行超声心动图检查。确定血压参数异常的患病率，并评估其与SCD严重程度和终末器官损伤指标的关系。结果 67例患者有足够的ABPM数据。入组儿童年龄为13±4岁（40%为男性）。昼夜变化评估显示，81%的患者夜间收缩压下降异常，61%的患者夜间舒张压下降异常。昼夜模式异常与网织红细胞增多和超滤有关。19%（n = 13）的患者存在微量白蛋白尿，其中77%（n = 10）为女性（p = 0.014）。舒张压负荷和夜间下降异常与超滤有关，但与微量白蛋白尿无关。结论 通过ABPM检测到的SCD患者血压异常很普遍，可能是终末器官并发症的危险因素。需要进一步研究以确定这些关系背后的机制及其纵向变化。
