PSYCHOSOCIAL ADAPTATION OF 39 ADOLESCENTS WITH SEX-CHROMOSOME ABNORMALITIES

Objectives. Children with sex chromosome abnormalities (SCA) are known to be at increased risk for neuromotor, language, learning, and behav ioral problems, but little is known of psychosocial adaptation of SCA adolescents. This study was conducted to evaluate psychologic characte ristics of unselected SCA adolescents, including socialization, educat ional progress, separation from family, and incidence and severity of psychiatric disturbance. Methods. Thirty-nine propositi identified thr ough the screening of 40 000 consecutive Denver newborns, including bo ys with 47,XXY karyotypes and girls with 47,XXX, 45,X, and partial X m onosomy, or SCA mosaic karyotypes, have been followed longitudinally i nto adolescence. Twenty-seven siblings served as controls. Between 12 and 19 years of age, all participated in blind psychiatric interviews and were administered standardized intelligence and achievement tests. Results. SCA propositi demonstrated a mean IQ score 21 points lower t han that of control subjects. In addition, lower mean scores were seen on achievement test results as well as lower overall psychosocial ada ptation scores and increased incidence of psychiatric disturbance. Dep ression was the most frequent psychiatric diagnosis. Propositi were mo re likely to receive special education assistance in high school and w ere less likely to graduate from high school than were controls. Of th e three nonmosaic propositi groups, the 47,XXX girls demonstrated the poorest overall psychosocial adaptation and highest degree of psychiat ric disturbance. Mosaic girls were indistinguishable from control subj ects. Marked variability was found among all three nonmosaic groups, w ith some individuals in each group demonstrating relatively strong psy chosocial adaptation. Conclusions. The presence of nonmosaic sex chrom osome abnormality increases the risk for impeded cognitive skills, lea rning abilities, and psychosocial adaptation in adolescence. The facto rs that allow for stronger adaptation in some of these adolescents inc lude the presence of a stable and supportive family environment. The o utlook for adaptation in unselected SCA adults remains uncertain.