Hypogonadism and Sex Steroid Replacement Therapy in Girls with Turner Syndrome

J Pediatr Adolesc Gynecol. 2016 Dec;29(6):542-550. doi: 10.1016/j.jpag.2016.03.005. Epub 2016 Mar 25.

Abstract

Turner syndrome is the most common example of hypergonadotropic hypogonadism resulting from gonadal dysgenesis. Most patients present delayed, or even absent, puberty. Premature ovarian failure can be expected even if spontaneous menarche occurs. Laboratory markers of gonadal dysgenesis are well known. The choice of optimal hormone replacement therapy in children and adolescents remains controversial, particularly regarding the age at which therapy should be initiated, and the dose and route of estrogen administration. On the basis of a review of the literature, we present the most acceptable schedule of sex steroid replacement therapy in younger patients with Turner syndrome.

Keywords: Hypogonadism; Sex hormone replacement therapy (HRT); Sex steroid replacement therapy; Turner syndrome.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Age Factors
  • Child
  • Drug Administration Schedule
  • Estrogens / administration & dosage*
  • Female
  • Gonadal Steroid Hormones / administration & dosage*
  • Hormone Replacement Therapy / methods*
  • Humans
  • Hypogonadism / drug therapy*
  • Hypogonadism / etiology
  • Menarche
  • Turner Syndrome / complications
  • Turner Syndrome / drug therapy*

Substances

  • Estrogens
  • Gonadal Steroid Hormones