Day One: Key management points for young women with Turner syndrome described

2019-09-22T10:39:45+00:00September 19th, 2019|Growth disorders, News report, Paediatric endocrinology|

medwireNews: Oestrogen is key to the management of adolescents and young women with Turner syndrome, with hormone replacement therapy (HRT) generally preferable to the contraceptive pill, delegates were told at the 58th Annual ESPE Meeting in Vienna, Austria.

However, Melanie Davis, from University College London Hospital in the UK, also noted that patient preference, comorbidity, and the need for oral contraceptives should be taken in to account when treating these patients.

During the ESPE working group on Turner syndrome, Davis gave a gynaecologist’s perspective on endocrine management of these patients during late adolescence and early adulthood, ie, below the age of 25 years, when they are initiating oestrogen replacement treatment for the induction of puberty.

She explained that the management of adolescent girls with Turner syndrome often depends on when they present and who they are treated by: those with short stature and absence of puberty are generally treated by paediatricians with ethinyloestradiol, whereas those with secondary amenorrhoea are typically treated by gynaecologists and put straight on to a combined oral contraceptive pill, which is “not a good idea if they haven’t completed pubertal induction.”

Indeed, Davis believes that HRT is preferable to the combined oral contraceptive pill for a number of reasons, including greater improvements in uterine volume relative to no oestrogen use.

In addition, she said that previous data has shown that HRT is associated with reductions in blood pressure and increases in bone mineral density (BMD), whereas the pill does the opposite. Nonetheless, she pointed out that both treatments are better than none at all.

Davis is now planning a randomised controlled trial comparing the effect of the combined oral contraceptive pill with that of HRT, delivered both orally and transdermally, on BMD among almost 500 young women with Turner syndrome in the UK. She said the study will also look at the impact on quality of life and cardiovascular markers, and will include a long-term observational follow-up period.

Alongside the importance of oestrogen Davis discussed the need for progesterone, which she said is “essential” for the prevention of endometrial hyperplasia. Once introduced, there is limited data with mixed results on whether cyclical or continuous therapy is best. Therefore, in young women “we should follow physiology” unless there are special circumstances such as disabilities or severe learning difficulties, Davis remarked.

She added that when using oestrogen with progestogen, the preferred delivery method should be transdermal because of the relative contraindications to the oral route, such as increased thrombotic risk, obesity and migraine.

Monitoring HRT in young women with Turner syndrome should, according to Davis, involve feedback from the patients, along with annual blood pressure and weight measurement, and BMD testing every 3–5 years. By contrast, she said that gynaecology examinations are not needed unless there are symptoms, and blood tests for oestrogen levels are of little value in a monitoring situation because results vary depending on when the patch is applied.

Davis concluded by emphasizing the importance of the transition phase in these patients and pleading with the paediatric endocrinologists in the room to “please collaborate with your adult endocrinologist and your gynaecologist.”

By Laura Cowen

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