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

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

Author information

1
Genetikum, Neu-Ulm, Germany.
2
Department of Neurobehavioral Genetics, Trier University, Trier, Germany.
3
Department of Biological and Clinical Psychology, Trier University, Trier, Germany.
4
Department of Child and Adolescent Psychiatry and Psychotherapy, Homburg, Saar, Germany.
5
Practice of Human Genetics, Homburg, Saar, Germany.
6
Institute of Human Genetics, Homburg, Saar, Germany.

Abstract

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.

PMID:
30070765
DOI:
10.1002/ajmg.a.40477

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