Previous work (Bath et al. 2001) has shown that while pubertal induction in Turner syndrome (TS) is fairly standard, post-induction oestrogen replacement is more variable. To identify current practice and to inform the design of two proposed randomised controlled trials (RCTs), a questionnaire was devised on behalf of the British Society for Paediatric Endocrinology and Diabetes for its members, members of the British Society for Paediatric and Adolescent Gynaecology and clinicians running Adult Turner Clinics. Sixty-four consultants (48 paediatric, 16 adult) currently seeing TS patients have responded.
Pubertal induction: Of the 57 respondents (48 paediatric, 9 adult) prescribing oestrogen for pubertal induction, 51 use oral Ethinylestradiol, 3 use other forms of oestrogen (patch/gel/oral) and 2 use both. The distribution of ages at which start of treatment is preferred is 10 years (2), 11 years (6), 12 years (28), 13 years (11) and 14 years (5). Comments were invited on a proposed RCT comparing oral Ethinylestradiol with transdermal patch. Favoured outcome measures were, in order of preference, breast development, uterine length/shape and patient preference. Fifty respondents expressed interest in participating with an estimated 150 eligible patients.
Post-induction treatment: Of the 61 respondents (45 paediatric, 16 adult) prescribing oestrogen after puberty, 52 use a combined oral contraceptive pill (OCP), 34 use oral oestrogen/progesterone and 18 use transdermal patch. Reasons include familiarity, patient convenience and acceptability (OCP), continuous and/or natural oestrogen (oral oestrogen/progesterone), physiological and continuous (transdermal). Comments were invited on a proposed RCT comparing OCP, oral HRT and patch. Thirteen respondents made alternative suggestions including oestrogen gel and alternative preparations/doses. Forty-six respondents expressed interest in participating with an estimated 150 eligible patients. In conclusion, most UK clinicians still use oral Ethinylestradiol to induce puberty in girls with TS while oral, rather than transdermal, oestrogen appears to be preferred after puberty. Thus, the benefits of more physiological preparations proposed by studies in post-menopausal women and small numbers of TS patients have not been translated into current UK practice and are unlikely to be, without well designed and rigorously conducted clinical trials, for which there is apparent widespread support.
05 - 07 Nov 2008
British Society for Paediatric Endocrinology and Diabetes