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Women with Turner syndrome are both estrogen and androgen deficient - the impact of hormone replacement therapy.

CONTEXT
Turner syndrome women suffer from hypergonadotropic hypogonadism, causing a deficit in the gonadal hormone secretion. As a consequence, Turner syndrome women are treated with estrogen from the age of 12 years old, and later in combination with progesterone. However, androgens have been given less attention.


OBJECTIVE
To assess sex hormone levels in Turner syndrome women, both treated and non-treated with hormone replacement therapy (HRT), and investigate the impact of HRT on sex hormone levels.


DESIGN AND PARTICIPANTS
Ninety-nine Turner syndrome participants were followed three times from August 2003 to February 2010. Seventeen Turner syndrome women were lost during follow-up. Control group 1 consisted of sixty-eight healthy age-matched control women seen once during this period. Control group 2 consisted of 28 young, eumenorrheic women sampled 9 times throughout the same menstrual cycle.


SETTING
Aarhus University Hospital.


MAIN OUTCOME MEASURES
Serum concentrations of follicle stimulating hormone (FSH), luteinizing hormone (LH), 17β-estradiol, estrone sulphate, DHEAS, testosterone, free androgen index, androstenedione, 17-OH progesterone and sex hormone-binding globulin (SHBG).


RESULTS
All androgens, 17-OH progesterone and SHBG were 30-50% lower in Turner syndrome women compared with controls (p <0.01). FSH, LH and estrone sulphate were more than doubled in Turner syndrome women compared with controls (p<0.02). Using principal component analysis, we describe a positive correlation between Turner syndrome women receiving HRT, elevated levels of SHBG and decreased levels of androgens.


CONCLUSION
The sex hormone profile in Turner syndrome reveals a picture of androgen deficiency, aggravated further by HRT. Conventional HRT does not normalize estradiol levels in Turner syndrome.