Women With Turner Syndrome Are Both Estrogen and Androgen Deficient: The Impact of Hormone Replacement Therapy

Author:

Viuff Mette Hansen12ORCID,Just Jesper1ORCID,Brun Sara2ORCID,Dam Tine Vrist3ORCID,Hansen Mette3,Melgaard Lars4,Hougaard David M4ORCID,Lappe Michael5,Gravholt Claus Højbjerg12ORCID

Affiliation:

1. Department of Molecular Medicine, Aarhus University Hospital , 8200 Aarhus , Denmark

2. Department of Endocrinology and Internal Medicine, Aarhus University Hospital , 8200 Aarhus , Denmark

3. Department of Public Health, Aarhus University , 8000 Aarhus , Denmark

4. Danish Center for Neonatal Screening, Clinical Mass Spectrometry, Statens Serum Institut , 2300 Copenhagen , Denmark

5. CONNECT, Aarhus University Hospital , 8200 Aarhus , Denmark

Abstract

Abstract Context Women with Turner syndrome (TS) suffer from hypergonadotropic hypogonadism, causing a deficit in gonadal hormone secretion. As a consequence, these women are treated with estrogen from the age of 12 years, and later in combination with progesterone. However, androgens have been given less attention. Objective To assess sex hormone levels in women with TS, both those treated and those nontreated with hormone replacement therapy (HRT), and investigate the impact of HRT on sex hormone levels. Methods At Aarhus University Hospital, 99 women with TS were followed 3 times from August 2003 to February 2010. Seventeen were lost during follow-up. Control group 1 consisted of 68 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. Serum concentrations of follicle-stimulating hormone (FSH), luteinizing hormone (LH), 17β-estradiol, estrone sulfate, DHEAS, testosterone, free androgen index, androstenedione, 17-OH progesterone, and sex hormone–binding globulin (SHBG) were analyzed. Results All androgens, 17-OH progesterone, and sex hormone–binding globulin (SHBG) were 30% to 50% lower in TS compared with controls (P < 0.01). FSH, LH, and estrone sulfate were more than doubled in women with TS compared with controls (P < 0.02). Using principal component analysis, we describe a positive correlation between women with TS receiving HRT, elevated levels of SHBG, and decreased levels of androgens. Conclusion The sex hormone profile in TS reveals a picture of androgen deficiency, aggravated further by HRT. Conventional HRT does not normalize estradiol levels in TS.

Funder

Novo Nordisk Foundation

Familien Hede Nielsens Fond

Independent Research Fund

Publisher

The Endocrine Society

Subject

Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,Endocrinology, Diabetes and Metabolism

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