Dykens E, Ort S, Cohen I, Finucane B, Spiridigliozzi G, Lachiewicz A, Reiss A, Freund L, Hagerman R, O'Connor R
Yale Child Study Center, New Haven, Connecticut, USA.
J Autism Dev Disord. 1996 Jun;26(3):287-301. doi: 10.1007/BF02172475.
We conducted two multicenter studies on adaptive trajectories and profiles in males with fragile X syndrome. Study 1 longitudinally assessed 29 males ages 1-20 years using age-equivalent scores from the Vineland Adaptive Behavior Scales. Fragile X boys ages 1-10 years showed significant gains in adaptive skills from first to second testing; males ages 11-20 years were stable in their adaptive development. Study 2 cross-sectionally examined 132 males ages 1-20 years. Significant age-related gains were found in boys ages 1-10, particularly in preschool children. Subjects ages 11-20 showed increased variability and nonsignificant relations between age and adaptive skills. Preliminary findings from 26 young adults with fragile X syndrome ages 21-40 years showed stable age-equivalent adaptive scores during these years. Relative strengths in daily living skills and weaknesses in communication were only evident among older subjects. Significant relations were found between adaptive behavior standard scores and IQ; these two scores also showed age-related declines that likely parallel one another. Findings are related to adaptive features in other genetic syndromes, and to directions for future adaptive behavior research.
我们针对脆性X综合征男性患者的适应性轨迹和特征开展了两项多中心研究。研究1使用《文兰适应行为量表》的年龄当量分数,对29名年龄在1至20岁的男性进行了纵向评估。1至10岁的脆性X男孩在首次测试到第二次测试期间,适应技能有显著提高;11至20岁的男性在适应性发展方面保持稳定。研究2对132名年龄在1至20岁的男性进行了横断面研究。发现1至10岁的男孩有显著的与年龄相关的进步,尤其是学龄前儿童。11至20岁的受试者年龄与适应技能之间的变异性增加且关系不显著。对26名年龄在21至40岁的脆性X综合征青年成人的初步研究结果显示,这些年里年龄当量适应分数保持稳定。日常生活技能方面的相对优势和沟通方面的劣势仅在年龄较大的受试者中明显。适应行为标准分数与智商之间存在显著关系;这两个分数还显示出与年龄相关的下降,且可能相互平行。研究结果与其他遗传综合征的适应性特征以及未来适应行为研究的方向有关。