Women with Turner syndrome are both estrogen and androgen deficient—the impact of hormone replacement therapy
Open accessAbstract
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.
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Many young women with Turner syndrome benefit from a higher dose of estrogen than their older peers, exceeding 2 mg E2 orally or 50 mcg transdermally.
“Many young women with TS benefit from a higher dose of estrogen than their older peers, that is, exceeding 2 mg E2 orally or 50 mcg TD.”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [303]
Higher estrogen doses are particularly important for achieving maximal uterine growth and development as options for fertility treatment increase.
“This is particularly important for achieving maximal uterine growth and development as options for fertility treatment increase.”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [303]
Viuff et al. reported that E2 concentrations during hormone replacement therapy in Turner syndrome were comparable to controls only at early follicular phase, suggesting that the current regimen does not fully normalize E2 concentrations in Turner syndrome.
“Viuff et al. reported that E2 concentrations during HRT in TS were comparable to controls only at early follicular phase suggesting that the current regimen does not fully normalize E2 concentrations in TS.”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [303]
It is not feasible to mimic the physiological cyclic variations of the adult woman with intact ovarian function.
“As previously discussed, it is not feasible to mimic the physiological cyclic variations of the adult woman with intact ovarian function so all risks and benefits of options need to be considered.303”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [303]
Androgen concentrations are 30%-50% lower in women with TS than in controls.
“Viuff et al. showed that androgen concentrations are 30%-50% lower in women with TS than in controls.303”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [303]
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- Authors
- Viuff, M. H., Just, J., Brun, S., Dam, T. V., Hansen, M., Melgaard, L., Hougaard, D. M., Lappe, M., & Gravholt, C. H.
- Affiliations
- Aarhus University Hospital (DK)
- Aarhus University (DK)
- Statens Serum Institut (DK)
- CONNECT, Aarhus University Hospital , 8200 Aarhus , Denmark
- Corresponding author
- Viuff, Mette Hansen
- Just, Jesper
- ORCID
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2022-03-18
- Volume
- 107
- Issue
- 7
- Pages
- 1983-1993
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W4220716885
- Cited by
- 35
- References
- 42
- Citations by year
- 2026: 3 · 2025: 13 · 2024: 11 · 2023: 6 · 2022: 2
- FWCI
- 4.85
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model (vor)
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Novo Nordisk Foundation (NNF15OC0016474, NNF20OC0060610)
- Familien Hede Nielsens Fond
- Independent Research Fund (0134-00406A)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal and reproductive studies
- Keywords
- Turner syndrome, hormone replacement therapy, androgen deficiency, estrogen deficiency, sex hormone levels, hypergonadotropic hypogonadism, sex hormone-binding globulin, estradiol, testosterone, dehydroepiandrosterone sulfate, androstenedione, luteinizing hormone, follicle-stimulating hormone, estrone sulfate
- MeSH terms
- Androgens, Estradiol, Estrogens, Female, Follicle Stimulating Hormone, Humans, Luteinizing Hormone, Progesterone, Sex Hormone-Binding Globulin, Gonadal Steroid Hormones, Testosterone, Turner Syndrome, Hormone Replacement Therapy
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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