Neurodevelopmental Deficits Among Infants and Toddlers with Sickle Cell Disease
Neurodevelopmental Deficits Among Infants and Toddlers with Sickle Cell Disease
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DOI：
10.1097/dbp.0b013e31829c3c48
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发表时间：
2013-07-01
影响因子：
2.4
通讯作者：
Gordeuk, Victor
中科院分区：
文献类型：
--
作者：
Glass, Penny;Brennan, Tara;Gordeuk, Victor
目的：神经发育缺陷是镰状细胞病的严重并发症之一。然而，很少有研究前瞻性地评估SCD儿童的神经发育缺陷。我们分析了80名患有SCD的婴儿和幼儿的基线神经发育数据，以确定与早期发育延迟相关的主要疾病相关事件和社会人口学风险因素。方法：这是一项为期4年的混合横断面/纵向研究的基线数据分析。患有SCD(任何基因型)的3.5岁或以下的足月儿童符合条件。在9、15、21、30和40个月时进行神经发育评估(Bayley II)。结果：有明显的神经发育缺陷：17.5%的人在贝利精神指数或运动指数上得分低于平均值。在控制了社会经济地位(SES)、性别、肺炎/急性胸部综合征和血红蛋白浓度后，那些经历过血管闭塞性疼痛发作的人发生显著发育迟缓的优势比是后者的9倍。男性也是发育迟缓的危险因素。结论：SCD儿童存在早期认知和运动迟缓，经历过疼痛危机的儿童患病率较高。男性易感性的增加与其他高危人群是一致的，但在SCD研究中以前没有涉及到。此外，这些延迟不能用较低的SES来充分解释。SCD儿童严重的发育迟缓可能不会被初级保健实践、医学专科诊所或父母认识到。对患有慢性疾病的儿童进行常规神经发育评估的重要性是显而易见的。
Objective:Neurodevelopmental deficits are among the serious complications of sickle cell disease (SCD). However, few studies have prospectively evaluated neurodevelopmental deficits in very young children with SCD. We analyzed baseline neurodevelopmental data from a cohort of 80 infants and toddlers with SCD to identify primary disease-related events and sociodemographic risk factors associated with early developmental delay.Methods:This is an analysis of baseline date of a 4-year mixed, cross-sectional/longitudinal study. Full-term children at age 3.5 years or younger with SCD (any genotype) were eligible. Neurodevelopmental evaluations (Bayley II) were conducted at ages 9, 15, 21, 30, and 40 months. Demographics, hematologic variables, and medical events were obtained.Results:Significant neurodevelopmental deficits were evident: 17.5% scoring >2SD below the mean on Bayley Mental Index or Motor Index. Odds ratio of significant developmental delay was >9 times more likely among those who had experienced vaso-occlusive pain episodes, after controlling for socioeconomic status (SES), gender, pneumonia/acute chest syndrome, and hemoglobin concentration. Male gender was also a risk factor for developmental delay.Conclusions:Early cognitive and motor delays were present in young children with SCD, with higher prevalence among those who had experienced pain crises. Increased vulnerability of male gender is consistent with other at-risk populations but has not been previously addressed in SCD research. Furthermore, these delays are not sufficiently explained by lower SES. Significant developmental delay in children with SCD may go unrecognized by primary care practices, medical specialty clinics, or parents. The importance of routine neurodevelopmental assessment for children with chronic medical conditions is clear.