Quality of Life in Turner Syndrome after Growth Hormone Treatment
© 2005 Prime Health Consultants, Inc.
Growth hormone (GH) treatment reliably increases adult height in women with Turner syndrome (TS). Less clear are the benefits of increased height on indices of adult quality of life (QOL). Carel and colleagues assessed clinical predictors of QOL in young adult women with TS enrolled in France’s national GH-treatment registry.
In 2001, there were 891 women 18 years or older who were eligible for the study (568 completed the postal questionnaire survey, 250 did not respond, and the remaining 73 were either lost to follow-up, unable to complete the forms because of low IQ, or were deceased). The mean age at initiation of GH treatment for respondents and nonrespondents was 12 + 2.5 years. Treatment lasted on average 4.7 years and ended when the girls were 16.7 + 1.6 years. The questionnaire was completed approximately 6 years later at age 22.6 + 2.6 years. Mean adult height was 150.9 + 5.6 cm and 148.2 + 6.7 cm for respondents and nonrespondents, respectively (P<0.005). Quality of life (assessed by, the French version of the Medical Outcome Study Short Form 36 [SF-36], the General Health Questionnaire 12 [GHQ-12]) questions examined demographic characteristics, sexual history, and expectations of growth benefits from GH.
The TS group did not differ from population norms (women 18 to 24 yrs) in their scores on the SF-36, a generic measure of health-related QOL. Scores on the GHQ-12, a measure of psychological well-being were lower (ie, better function) in the TS group compared to norms. In statistical analyses controlling for demographic characteristics, adult height was not associated with QOL scores. Patients with the highest expectations for growth-promoting effects of GH reported the lowest QOL. Otological impairment was reported in 26% of the participants, and this was associated with poorer scores on multiple QOL scales. “Induction of puberty after 15 years” was associated with more negative perceptions of health, which represented a more circumscribed negative effect than that observed for hearing problems. Variables not found to be statistically related to QOL scale scores included karyotype, associated dysmorphic features, sexual intercourse experience, thyroid dysfunction, or self-reported visual problems.
Editor’s Comment: This registry-based study is the first to examine the health-related QOL of young adult women with TS who received treatment with GH. Adult height (for 95% of the sample) ranged from 139 to 161 cm. Although mean adult height reflected a 6 to 8 cm gain above historical data for French patients, relative height for the participating portion of this cohort was still short (—2.2 htSD), and the adult height for nonparticipants was even shorter (—2.7ht SD). Despite this modest benefit of GH treatment, the authors emphasize that the QOL of participants was on par with norms for the general French population. Given that a contemporaneous cohort of untreated women with TS was not available for comparison, and that adult height was not associated with scores on QOL measures, the authors speculate that “a beneficial effect of GH treatment irrespective of adult height attained cannot be ruled out.” One might contemplate if the act of visiting a endocrinology clinic at regular intervals and quite likely of meeting other girls with TS, has a salutary effect on QOL that is independent of the growth-promoting medical intervention.
In examining the association between clinical characteristics and QOL, otological rather than auxological status predicted QOL. It is unlikely that the pattern of these associations would change with more complete participation of the cohort in the survey; there is no reason to expect that the addition of nonresponders would influence the correlation between height and QOL indices insofar as there was adequate range in adult heights among the responders to detect correlations, if they existed.
A separate follow-up study of young adult women with TS suggests that the transition from pediatric to adult medical care is suboptimal, with a significant minority not receiving regular healthcare and not continuing with estrogen replacement.1 For these reasons, multidisciplinary follow-up clinics which facilitate transition in medical care should be considered for TS, as has been described for individuals with congenital adrenal hyperplasia.2
David E. Sandberg, PhD
References - (linked to 
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- Verlinde F, Massa G, Lagrou K, et al. Health and psychosocial status of patients with turner syndrome after transition to adulthood: the Belgian experience. Horm Res. 2004;62:161-167.
- Kruse B, Riepe FG, Krone N, et al. Congenital adrenal hyperplasia - how to improve the transition from adolescence to adult life. Exp Clin Endocrinol Diabetes. 2004;112:343-355.