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Turner Syndrome pp 93–122Cite as

Estrogen Replacement in Turner Syndrome

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Abstract

Most girls with Turner syndrome have hypergonadotropic hypogonadism and need hormonal replacement – for induction of puberty and later maintaining secondary sex characteristics, attaining peak bone mass, and uterine growth. The goals of replacement are to mimic normal timing and progression of physical and social development while minimizing risks. Treatment should begin at 11–12 years old, with a very low dose and increase over 2–3 years. Initiation with low doses of estradiol is crucial to preserve growth potential. Delaying estrogen replacement may be deleterious to bone and uterine health. We suggest transdermal estrogen and oral progestin and discuss other approaches. We also discuss linear growth, metabolic markers, neurocognition, socialization, and bone and uterine health as related to hormonal replacement.

Once progestin is added, many women prefer the ease of use of a pill containing both an estrogen and progestin. The risks and benefits of different preparations, with examples, are discussed.

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Authors and Affiliations

  1. University of California, San Diego & Rady Children’s Hospital, San Diego, CA, USA

    Karen O. Klein

  2. The University of Chicago Pritzker School of Medicine, Chicago, IL, USA

    Robert L. Rosenfield

  3. University of Virginia, Charlottesville, VA, USA

    Richard J. Santen

  4. Department of Pediatrics and Pediatric Endocrinology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland

    Aneta M. Gawlik

  5. The Cincinnati Center for Pediatric and Adult Turner Syndrome Care, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Philippe Backeljauw

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

    Claus H. Gravholt

  7. Department of Molecular Medicine, Aarhus University Hospital, Aarhus, Denmark

    Claus H. Gravholt

  8. Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark

    Claus H. Gravholt

  9. Albert Schweitzer Hospital Dordrecht and Sophia Children’s Hospital Rotterdam, Rotterdam, The Netherlands

    Theo C. J. Sas

  10. Nemours Children’s Health System, Jacksonville, FL, USA

    Nelly Mauras

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  1. Karen O. Klein
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  7. Theo C. J. Sas
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  1. Division of Endocrinology, Seattle Children’s Hospital, University of Washington, Seattle, WA, USA

    Patricia Y. Fechner

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Klein, K.O. et al. (2020). Estrogen Replacement in Turner Syndrome. In: Fechner, P. (eds) Turner Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-34150-3_5

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  • DOI: https://doi.org/10.1007/978-3-030-34150-3_5

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