An earlier age of onset of puberty may be beneficial for adult bone density in Turner syndrome and type of hormone therapy may have a varying impact on adult health, according to study findings published in The Journal of Clinical Endocrinology & Metabolism.
The majority of adolescents with Turner syndrome (approximately 85%) will experience delayed puberty and primary amenorrhea and require hormone therapy. After the onset of puberty, various types of maintenance estrogen replacement therapy are used to maintain secondary sex characteristics and overall well-being. The use of estrogen replacement therapy in Turner syndrome has also been linked to the normalization of raised liver enzymes and improved bone mineral density. In this study, researchers determined associations between age of induction of puberty and the type of estrogen therapy on adult health outcomes. The cohort included 799 women with Turner syndrome who were treated at a single clinic, and of this group, 624 had primary amenorrhea and 599 had accurate maintenance estrogen replacement therapy data.
The starting age for estrogen therapy was negatively correlated with adult bone density (spine: r, -0.22; hip: r, -0.20; P <.001). Women who used oral contraceptives had higher total cholesterol, low-density lipoprotein cholesterol, and triglycerides compared with other types of estrogen therapy. Blood pressure was also higher in women using oral contraceptive pills vs oral estrogens (estradiol valerate, conjugated equine) or transdermal estradiol (P ≤.001). In addition, the liver enzymes alanine transaminase, alkaline phosphatase, and gamma-glytamyl transpeptidase were elevated in women receiving transdermal estradiol compared with other groups (P ≤.001). Hemoglobin A1c was also elevated in transdermal estradiol users (P ≤.01).
“When considering our data and the propensity for women with [Turner syndrome] to develop a variety of adverse outcomes such as high blood pressure, [oral contraceptives are] relatively contraindicated as a form of [estrogen replacement therapy],” wrote the researchers. “While [transdermal estradiol] may be of benefit in terms of thrombosis risk, this has to be balanced against the finding of higher liver enzymes in this group, the long-term consequences of which require further exploration.”
Cameron-Pimblett A, Davies MC, Burt E, et al. Effects of estrogen therapies on outcomes in Turner Syndrome: assessment of induction of puberty and adult estrogen use [published online February 6, 2019]. J Clin Endocrinol Metab. doi:10.1210/jc.2018-02137