Psychological aspects of Turner syndrome

Turner syndrome (TS) is a sex-chromosome disorder, occurring in 1 in 2500 female births. The principal features of TS are short stature and dysfunctional gonads, resulting in a lack of sex hormones, incomplete pubertal development and impaired fertility. The aim of this paper is to review the literature on the psychological effects of TS. The main areas covered relate to well-being and psychopathology, self-esteem, social functioning, gender identity, partner relations and sexual functioning, coping, family aspects and clinical aspects of cognitive impairment. Research on the psychological effects of medical intervention is described, and the methods used for psychological and educational support are presented. Finally, methodological issues are discussed and areas for future research are proposed.