BEHAVIORAL CHARACTERISTICS OF CHILDREN WITH SICKLE CELL DISEASE

ABSTRACT Objective: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics. Methods: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability. Results: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%. Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.

摘要
研究目的：评估镰状细胞病（sickle cell disease, SCD）患儿的社会人口学与临床特征，及其行为特点。
研究方法：对4~10岁镰状细胞病患者的家长开展访谈，收集社会经济状况及其他健康相关信息，并采用长处和困难问卷（Strengths and Difficulties Questionnaire, SDQ）进行行为评估；临床数据从患儿病历中提取。本研究排除标准为：患儿接受羟基脲治疗、既往诊断为脑卒中、慢性脑病或智力障碍。
研究结果：共纳入45例患儿（女童19例，男童26例），中位年龄为7岁。镰状细胞病分型：血红蛋白SC病26例，血红蛋白SS病19例。社会经济阶层分布：D级占比24.4%，C2级占比44.4%，C1级占比28.9%，B2级占比2.2%。临床病史相关指标：急性胸综合征发生率为40%，输血治疗率为66.7%，住院率为82.2%。长处和困难问卷评估结果显示：88.9%的患儿存在临床行为影响（情绪子维度占比68.9%）；总分维度下，48.9%的患儿存在行为影响。未发现疾病严重程度与长处和困难问卷评分存在相关性。社会经济阶层层面分析：B2与C1阶层患儿中，21.4%存在总分维度的行为影响；而C2与D阶层患儿的该比例达61.3%。家庭户主受教育程度分析结果显示：户主至少完成初等教育的家庭中，39.3%的患儿存在行为影响；户主受教育程度更低的家庭中，该比例为64.7%。
研究结论：镰状细胞病患儿的行为影响发生率极高。C2及D阶层的患儿相较于B2与C1阶层患儿，更易出现行为影响。