Psychosocial well-being in Dutch adults with disorders of sex development Nita G.M. de Neve–Enthoven a , Nina Callens b , Maaike van Kuyk c , Jet H. van Kuppenveld c , Stenvert L.S. Drop d , Peggy T. Cohen–Kettenis e , Arianne B. Dessens d, ⁎ a Department of Child and Adolescent Psychiatry and Psychology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands b Department of Pediatrics, Division of Pediatric Endocrinology, University Hospital Ghent and Ghent University, Ghent, Belgium c Department of Medical Psychology, Radboud University Medical Center-Amalia Children's Hospital Nijmegen, Nijmegen, The Netherlands d Department of Pediatrics, Division of Pediatric Endocrinology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands e Department of Medical Psychology and Medical Social Work and Center of Expertise on Gender Dysphoria, VU University Medical Center Amsterdam, The Netherlands abstract article info Article history: Received 16 October 2015 Received in revised form 4 March 2016 Accepted 10 March 2016 Objective: Atypical sex development is associated with psychosocial vulnerability. We investigated psychosocial well-being in individuals with disorders of sex development (DSD) and hypothesized that psychosocial well-being was related to degree of genital atypicality at birth. Methods: 120 male (n = 16) and female (n = 104) persons with DSD, aged 14–60 years, participated in a follow-up audit on psychosocial well-being. They were stratified in: women with 1) 46,XY and female genitalia, 2) 46,XY or 46,XX and atypical genitalia, and 3) men with 46,XY and atypical genitalia. We used the Illness Cognition Questionnaire (ICQ), Checklist Individual Strength (CIS8R), TNO-AZL Quality of Life questionnaire (TAAQOL), Adult Self-Report (ASR), and the Rosenberg Self-Esteem Scale (RSES). Results: Data were compared to reference groups. Participants generally were coping well with DSD (ICQ). Women with DSD reported elevated levels of fatigue (CIS8R) and slightly more attention and memory problems (TAAQOL, ASR). Women with atypical genitalia reported more emotional and behavioral problems. On the ASR Rule-breaking Behavior and Antisocial Personality scales, these women had similar scores as reference men. Women with DSD reported a higher self-esteem (RSES). No differences in psychosocial well-being were found between men with DSD and reference men. Conclusion: Individuals with DSD across all diagnostic groups generally reported a good psychosocial well-being. The results further suggest involvement of prenatal androgens in the development of personality traits related to assertiveness and egocentricity. We recommend that individuals with a DSD and their families are involved in decision-making processes and have access to multidisciplinary care. © 2016 Elsevier Inc. All rights reserved. Keywords: Disorders of sex development (DSD) Psychosocial well-being Health related quality of life Self-esteem Emotional problems Fatigue 1. Introduction In individuals with disorders of sex development (DSD) the development of chromosomal, gonadal, and/or anatomic sex is atypical [1]. It is assumed that this incongruence puts them in a vulnerable position in society [2]. Current clinical management strategies therefore will include advice for early gender assignment, genital corrections, and hormonal treatments [1]. Lately, these early interventions have raised debate: it has been argued that they reflect society's intolerance to vari- ance in sex and gender and major decisions are made without consent of children themselves [3–6]. It has been suggested that postponement of gender assignment and genital surgery until the child is old enough to decide him/herself will benefit the child's well-being [7]. Randomized controlled comparison of the current treatment policy and the policy of delayed interventions is highly valued [8] but is difficult to conduct. The majority of parents living in Western countries choose gender assignment and genital surgery in early childhood [9–11]. Outcome studies on psychosocial well-being have been conducted. Due to differences in applied methodology and measures, findings are difficult to compare and show inconsistencies. These studies have mainly been carried out in females and focused on gender identity [12–18], sexual quality of life [19–23], and (psycho)sexual functioning [24–32], while studies on quality of life [33], social participation, self-esteem, and emotional problems are scarce. Studies addressing health related quality of life (HRQoL), emotional distress, and psychopathology in women with 46,XX congenital adrenal hyperplasia (CAH) revealed inconclusive outcomes, from reduced to a better HRQoL [20,34–36], and from no substantial emotional distress to increases in emotional problems [28, 37–40]. Women with complete androgen insensitivity syndrome (CAIS) Journal of Psychosomatic Research 83 (2016) 57–64 ⁎ Corresponding author at: Erasmus Medical Center Rotterdam – Sophia Children's Hospital, Department of Child and Adolescent Psychiatry and Psychology, P.O. Box 2060, Sh-1058, 3000, CB, Rotterdam, The Netherlands. E-mail address: a.b.dessens@erasmusmc.nl (A.B. Dessens). http://dx.doi.org/10.1016/j.jpsychores.2016.03.005 0022-3999/© 2016 Elsevier Inc. All rights reserved. Contents lists available at ScienceDirect Journal of Psychosomatic Research