Vitamin E Status and Total Body Water in Children and Adolescents with Sickle Cell Anemia

R. Williams-Hooker
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Abstract

Sickle Cell Disease (SCD) is a genetic disorder caused by a Hemoglobin gene mutation (HbS), which produces abnormal sickle he- moglobin that causes Red Blood Cells (RBC), to form rigid, sickle or holly-leaf-shaped cells rather than the typical round and flexible shape. These RBC’s are not able to pass through the vessels so less oxygen is transported to tissues, leading to vaso-occlusive pain cri - ses, organ damage, stroke, chronic anemia, decreased quality of life and early death. Nutritional concerns for children with SCD include weight loss and poor growth, delayed puberty, vitamin and mineral deficiencies and fluid imbalance. Several studies have shown that dietary intake falls below recommendations in SCD patients and that plasma levels of certain key vitamins, including vitamin E, are lower than reference values. Children with SCD have low to body water, which could be related, in part, to decreased intake. This study ex - plored vitamin E plasma status, vitamin E and fluid intake, and Total Body Water (TBW) status to determine if any of these variables were low. Each variable was also compared to pain scores to see if there was any correlation. Thirty African American children with SCD (15 female, mean age 12.03) participated in this study. Results showed that patients had decreased vitamin E and fluid intake, decreased corrected plasma vitamin E, and TBW below normal, however none of these factors was associated with pain medications. Further research needs to be conducted with a greater sample size and more pain variables.
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儿童和青少年镰状细胞性贫血患者的维生素E水平和全身水分
镰状细胞病(SCD)是一种由血红蛋白基因突变(HbS)引起的遗传性疾病,它产生异常的镰状血红蛋白,导致红细胞(RBC)形成坚硬的镰状或冬青叶状细胞,而不是典型的圆形和柔性形状。这些红细胞不能通过血管,因此输送到组织的氧气减少,导致血管闭塞性疼痛危机、器官损伤、中风、慢性贫血、生活质量下降和过早死亡。SCD儿童的营养问题包括体重减轻、发育不良、青春期延迟、维生素和矿物质缺乏以及体液失衡。几项研究表明,SCD患者的膳食摄入量低于推荐值,某些关键维生素(包括维生素E)的血浆水平低于参考值。患有SCD的儿童体内水分较低,这可能部分与摄入量减少有关。本研究探讨了维生素E血浆状态、维生素E和液体摄入量以及总身体水分(TBW)状态,以确定这些变量是否偏低。每个变量也与疼痛评分进行比较,看看是否有任何相关性。30名患有SCD的非裔美国儿童(女性15名,平均年龄12.03岁)参加了本研究。结果显示,患者维生素E和液体摄入量减少,血浆维生素E校正值降低,TBW低于正常水平,但这些因素与止痛药无关。进一步的研究需要更大的样本量和更多的疼痛变量。
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