Hormonal management of complete androgen insensitivity syndrome from adolescence onward

Horm Res Paediatr. 2011;76(6):428-33. doi: 10.1159/000334162. Epub 2011 Nov 29.

Abstract

Complete androgen insensitivity syndrome (CAIS) represents a main disorder of sex development. Women with CAIS may have their gonads removed before, during or after adolescence, thus requiring hormonal replacement therapy to induce puberty and/or maintain secondary sexual characteristics, to optimize bone mass accrual, and to promote physical and social well-being. Usually estrogens are used for this purpose, but formulations and doses should be better defined in multicentric prospective studies. Some women started testostosterone as hormonal replacement therapy, but this practice remains anecdotal. Bone health remains a crucial aspect in the management of persons with CAIS, but few sound data are available to guide clinical practice.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adolescent Development / drug effects*
  • Adult
  • Androgen-Insensitivity Syndrome / drug therapy*
  • Androgen-Insensitivity Syndrome / genetics
  • Androgen-Insensitivity Syndrome / physiopathology
  • Androgen-Insensitivity Syndrome / surgery
  • Bone Resorption / etiology
  • Bone Resorption / prevention & control
  • Estrogens / therapeutic use*
  • Hormone Replacement Therapy
  • Humans
  • Male
  • Mutation
  • Puberty / drug effects
  • Receptors, Androgen / genetics
  • Receptors, Androgen / metabolism
  • Testosterone / therapeutic use
  • Young Adult

Substances

  • Estrogens
  • Receptors, Androgen
  • Testosterone