Sex hormone replacement in Turner syndrome

Endocrine. 2012 Apr;41(2):200-19. doi: 10.1007/s12020-011-9569-8. Epub 2011 Dec 7.

Abstract

The cardinal features of Turner syndrome (TS) are short stature, congenital abnormalities, infertility due to gonadal dysgenesis, with sex hormone insufficiency ensuing from premature ovarian failure, which is involved in lack of proper development of secondary sex characteristics and the frequent osteoporosis seen in Turner syndrome. But sex hormone insufficiency is also involved in the increased cardiovascular risk, state of physical fitness, insulin resistance, body composition, and may play a role in the increased incidence of autoimmunity. Severe morbidity and mortality affects females with Turner syndrome. Recent research emphasizes the need for proper sex hormone replacement therapy (HRT) during the entire lifespan of females with TS and new hypotheses concerning estrogen receptors, genetics and the timing of HRT offers valuable new information. In this review, we will discuss the effects of estrogen and androgen insufficiency as well as the effects of sex HRT on morbidity and mortality with special emphasis on evidence based research and areas needing further studies.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adolescent Development / drug effects
  • Adult
  • Androgens / adverse effects
  • Androgens / therapeutic use*
  • Animals
  • Body Height / drug effects
  • Cardiovascular Diseases / etiology
  • Cardiovascular Diseases / prevention & control
  • Cognition Disorders / etiology
  • Cognition Disorders / prevention & control
  • Estrogens / adverse effects
  • Estrogens / therapeutic use*
  • Female
  • Hormone Replacement Therapy* / adverse effects
  • Human Growth Hormone / adverse effects
  • Human Growth Hormone / therapeutic use
  • Humans
  • Osteoporosis / etiology
  • Osteoporosis / prevention & control
  • Primary Ovarian Insufficiency / etiology
  • Primary Ovarian Insufficiency / prevention & control
  • Quality of Life
  • Recombinant Proteins / adverse effects
  • Recombinant Proteins / therapeutic use
  • Sexual Maturation / drug effects
  • Turner Syndrome / drug therapy*
  • Turner Syndrome / genetics
  • Turner Syndrome / physiopathology
  • Turner Syndrome / psychology
  • Young Adult

Substances

  • Androgens
  • Estrogens
  • Recombinant Proteins
  • Human Growth Hormone

Supplementary concepts

  • Juvenile osteoporosis