Hadeed Khaled, Hascoet Sébastien, Castex Marie-Pierre, Munzer Caroline, Acar Philippe, Dulac Yves
Department of Pediatric Cardiology, Children's Hospital, Toulouse, France.
Department of Pediatric Hematology, Children's Hospital, Toulouse, France.
Echocardiography. 2015 Aug;32(8):1285-90. doi: 10.1111/echo.12851. Epub 2014 Dec 3.
Sickle cell disease (SCD) is an inherited disorder characterized by recurrent painful crises with ischemia resulting from vascular occlusion. Adults with SCD have increased arterial stiffness and reduced flow-mediated dilation (FMD), due to impaired release of substances such as nitric oxide.
We aimed to assess the vascular properties of carotid and brachial arteries in children with SCD compared with a control group without cardiovascular risk factors.
Thirty patients with SCD, mean age 12.3 ± 4.5 years, were prospectively enrolled. A control group was made up of 30 age- and gender-matched healthy subjects. Carotid intima-media thickness (IMT), cross-sectional compliance (CSC), cross-sectional distensibility (CSD), diastolic wall stress (DWS), incremental elastic modulus (Einc), and FMD were determined in both groups.
There was no significant difference in FMD between the two groups (8.2 ± 5.0% in the SCD group vs. 9.3 ± 4.2% in the control group, P = 0.15). There was no significant correlation between FMD and age, hemoglobin, LDH level, or transcranial Doppler findings. CSD was significantly elevated in the SCD group (0.96 ± 0.44 vs. 0.59 ± 0.21, P = 0.0002), whereas DWS and Einc were significantly lower in the SCD group. CSC did not differ significantly between the two groups.
Children with SCD have no marked endothelial dysfunction or change in arterial stiffness. These manifestations may be related to disease severity and duration. Changes may become evident later in life as the disease progresses.
镰状细胞病（SCD）是一种遗传性疾病，其特征为因血管阻塞导致缺血而反复出现疼痛性危机。患有SCD的成年人由于一氧化氮等物质释放受损，动脉僵硬度增加，血流介导的血管舒张功能（FMD）降低。
我们旨在评估患有SCD的儿童与无心血管危险因素的对照组相比，其颈动脉和肱动脉的血管特性。
前瞻性纳入30例SCD患者，平均年龄12.3±4.5岁。对照组由30名年龄和性别匹配的健康受试者组成。测定两组的颈动脉内膜中层厚度（IMT）、横截面顺应性（CSC）、横截面扩张性（CSD）、舒张期壁应力（DWS）、增量弹性模量（Einc）和FMD。
两组之间的FMD无显著差异（SCD组为8.2±5.0%，对照组为9.3±4.2%，P = 0.15）。FMD与年龄、血红蛋白、乳酸脱氢酶水平或经颅多普勒检查结果之间无显著相关性。SCD组的CSD显著升高（0.96±0.44对0.59±0.21，P = 0.0002），而SCD组的DWS和Einc显著降低。两组之间的CSC无显著差异。
患有SCD的儿童没有明显的内皮功能障碍或动脉僵硬度变化。这些表现可能与疾病严重程度和病程有关。随着疾病进展，这些变化可能在以后的生活中变得明显。
