Behavioral and psychological features in girls and women with triple-X syndrome

Am J Med Genet A. 2018 Nov;176(11):2284-2291. doi: 10.1002/ajmg.a.40477. Epub 2018 Aug 2.


Triple-X syndrome is a common sex chromosome aneuploidy, which appears in 1 out of 1,000 females. The aim of our study was to describe the behavioral features of a large group of girls and women with triple-X in comparison to a control group. A total of 72 subjects with triple-X and 69 subjects of an age-matched control group were included. Psychological and behavioral questionnaires were allocated to three age groups, representing a range of ages from young childhood to adulthood. Regarding the females between 4 and 7 years of age, we found significant differences for social problems, attention problems, and school performance. For the age group 8-17 years, we found larger significant differences for the majority of the scales listed in the child behavior checklist. The most significant differences (p < .001) were from total behavior problems, internalizing problems, and four other scales. Young females with triple-X have significantly lower general self-esteem, especially concerning school and family. In the adults, there were significant differences concerning psychological symptoms and distress, with higher scores in the triple-X subjects. Regardless, their mean scores were still in the normal range. We did not find clinical evidence for more than 50% of the triple-X females in any age group, indicating that approximately half of them do not have behavioral problems, and that more than 60% do not differ in their competence from the control group. However, our findings suggest that triple-X influences mental health and the overall well-being of the individuals across their whole life spans.

MeSH terms

  • Adolescent
  • Adult
  • Behavior*
  • Case-Control Studies
  • Checklist
  • Child
  • Child Behavior
  • Child, Preschool
  • Chromosomes, Human, X
  • Female
  • Humans
  • Self Concept
  • Sex Chromosome Aberrations
  • Sex Chromosome Disorders of Sex Development / psychology*
  • Trisomy
  • Young Adult

Supplementary concepts

  • Triple X syndrome