Longitudinal changes in intellectual development in children with Fragile X syndrome
Longitudinal changes in intellectual development in children with Fragile X syndrome
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DOI：
10.1007/s10802-008-9223-y
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发表时间：
2008-08-01
影响因子：
3.6
通讯作者：
Reiss, Allan L.
中科院分区：
文献类型：
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作者：
Hall, Scott S.;Burns, David D.;Reiss, Allan L.
采用结构方程模型（SEM）对145对学龄儿童的智力功能发展进行了研究。每对包括一个患有脆性X综合征（FXS）的孩子和一个未受影响的兄弟姐妹。所有对儿童进行了评估韦氏儿童智力量表第三版（WISC-III）在时间1和80对儿童接受了第二次评估，在时间2约4年后。与未受影响的兄弟姐妹相比，FXS儿童在两个时间点的WISC所有分测验中获得的正确分数百分比显著较低。在第一次和第二次评估之间的时间内，未受影响的儿童的智力发育年速率比FXS儿童快约2.2倍。脆性X智力低下蛋白（FMRP）水平与FXS儿童在两个时间点的智力得分高度相关（r分别为0.55和0.64）。然而，当性别，年龄和评估之间的时间作为协变量包括在结构方程模型中，FMRP仅占5%的方差在智力得分在时间1和13%的方差在时间2。这项研究的结果表明，较慢的学习速度有助于在FXS儿童中观察到的标准化智商分数低和下降。
Structural equation modeling (SEM) was used to examine the development of intellectual functioning in 145 school-age pairs of siblings. Each pair included one child with Fragile X syndrome (FXS) and one unaffected sibling. All pairs of children were evaluated on the Wechsler Intelligence Scale for Children-Third Edition (WISC-III) at time 1 and 80 pairs of children received a second evaluation at time 2 approximately 4 years later. Compared to their unaffected siblings, children with FXS obtained significantly lower percentage correct scores on all subtests of the WISC at both time points. During the time between the first and second assessments, the annual rate of intellectual development was approximately 2.2 times faster in the unaffected children compared to the children with FXS. Levels of the fragile X mental retardation protein (FMRP) were highly associated with intellectual ability scores of the children with FXS at both time points (r=0.55 and 0.64 respectively). However, when gender, age, and the time between assessments were included as covariates in the structural equation model, FMRP accounted for only 5% of the variance in intellectual ability scores at time 1 and 13% of the variance at time 2. The results of this study suggest that slower learning contributes to the low and declining standardized IQ scores observed in children with FXS.