Psychosocial Functioning after Discontinuation of Long-Term Growth Hormone Treatment in Girls with Turner Syndrome

Abstract

It is common practice in the case of Turner syndrome (TS) to treat short stature with GH treatment and to induce puberty with estrogens at an age as close to normal puberty as possible. This approach in most cases leads to a height in the normal range in childhood, adolescence, and adulthood in TS. Little data is available, however, on its effect on psychosocial functioning. In the present study, we evaluated psychosocial functioning in a group of 50 women with TS, after reaching final height in two multicenter GH trials. Thirty-six girls participated in a randomized dose-response study from mean (SEM) age 6.8 (0.4) years, and 14 girls participated in a frequency-response study from age 13.2 (0.4) years. After discontinuation of long-term GH treatment, these 50 girls were evaluated for psychosocial functioning at a mean age of 18.8 (0.3) years. GH was given in a dosage of 4 IU/m²/day (∼0.045 mg/kg/day), 6 IU/m²/day, or 8 IU/m²/day. After a mean GH treatment duration of 7.1 (0.4) years, mean final height (ref. normal girls) was FH1.2 (0.2) SD score. Behavioral problem scores (Achenbach) of the TS women were comparable to normal Dutch peers. Although self-perception (Harter total scale: p < 0.01), and bodily attitude (Baardman: p < 0.05) was significantly less positive than for their normal Dutch peers, we found no evidence of depression. TS women rated their family functioning higher than their Dutch peers (p < 0.0001), and had a slightly different coping pattern. These results show that even after reaching a height in most cases within the normal range and puberty induction at a pubertal age, some women with TS still experience psychosocial problems. It is likely, however, that GH and estrogen treatment improved psychosocial functioning. Long-term follow-up of these GH-treated patients will allow an evaluation of their life achievements.

Psychosocial Functioning, Self-Perception and Body Image and Their Auxologic Correlates in Growth Hormone and Oestrogen-Treated Young Adult Women with Turner Syndrome

“…Our findings are globally in line with the YASR data reported by van Pareren et al [13] revealing comparable behavioural problem scores between TS women and the general population. However, their results were only based upon the broad-band scales.…”

“…Regarding self-perception, TS patients had self-perception scores comparable to the reference group on the majority of subscales except for social acceptance, romantic relations and sense of humour, globally in line with the Dutch results [13], but somewhat in contrast to other previous studies reporting impaired self-esteem and self-confidence [4, 6, 27, 29]. Problems in social relationships, particularly in contact with the opposite sex, as reflected by a lesser number of intimate relationships or marriages, later psychosexual milestones, problems with social cues have repeatedly been reported [4,5,6,7,8,9, 18].…”

“…Although it has been well-established that adults with TS are susceptible to a large range of medical and psychosocial problems [2, 3], psychological studies in adult women with TS report a large variability regarding psychosocial adaptation and functioning [4,5,6,7,8,9,10]. Moreover, the effects of height and height gain from growth hormone (GH) treatment on psychosocial functioning still remain unclear [11,12,13] and the effects of oestrogen replacement therapy have only been studied on self-concept and cognitive abilities in adolescents [14,15,16]. Recent studies suggest that adult height or height gain during GH therapy is not associated with quality-of-life scores, whereas older age at menarche and the lack of sex hormone replacement during adult life are major determinants of psychological well-being [17, 18].…”

Ambivalent Ambiguity? : A study of how women with 'atypical' sex development make sense of female embodiment

“…Such studies have shown that a delayed induction of puberty may have long-lasting effects on sex life (Carel et al, 2006) and that, even though height and pubertal treatments result in what is considered 'normal' height and puberty induction at a 'normal' age, the women affected may still present psychological difficulties because of the treatment (Carel et al, 2005;van Pareren et al, 2005). It has also been reported that Canadian girls and young women with TS score significantly lower when measuring general, social, and appearance-related self-esteem, but do not differ from women without Turner Syndrome when it comes to performance-related self-esteem (Cragg and Lafreniere, 2010).…”

O hormônio de crescimento na síndrome de Turner: dados e reflexões

“…Quanto ao momento apropriado para início da terapia de substituição hormonal ovariana, ainda que existam evidências para postergá-la com ganho adicional na altura pelo retardo do fechamento epifisário, a instituição de GHr por período suficientemente prolongado, antecedendo a idade cronológica de 12 anos, aparentemente não modifica, de modo significativo, o prognóstico estatural (24,25).…”