Review of recent outcome data of disorders of sex development (DSD): Emphasis on surgical and sexual outcomes

Peter Lee, Justine Schober, Anna Nordenström, Piet Hoebeke, Christopher P. Houk, Leendert Looijenga, Gianantonio Manzoni, William Reiner, Christopher Woodhouse

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

This paper is a review of some of the recent publications regarding outcome of DSD patients, with an emphasis upon surgical and sexual outcomes. Currently available outcome studies of patients with DSDs have limitations because of multiple factors, including lack of representative patient sampling, and lack of adequate information concerning both medical and surgical care, and psychological, social and family support. The most frequent reports involve females with 21-α-hydroxylase deficiency congenital adrenal hyperplasia (CAH). This most common form of DSD, if one excludes hypospadias and cryptorchidism, is an excellent example of a form of DSD in which all aspects of outcome, regarding surgery, sexual functionality and sensitivity, psychological input and endocrine hormonal therapy, carry a major role. The goals of therapy include a surgical outcome with a good cosmetic appearance and functionality with potential for sexual intercourse with sufficient sensitivity for satisfactory responsiveness. Endocrine replacement therapy should provide a normal adrenal hormonal milieu, while sex steroid therapy may be indicated. Psychological care should be provided from birth with gradual transition primarily to the patient, including basic counseling with full disclosure, although adjustment depends upon the patient's personality and parents' abilities and acceptance. Among forms of DSD involving gonadal insufficiency, hormonal replacement therapy should provide physiologic levels. Among females, estrogen therapy enhances healing after feminizing surgery and is required from puberty throughout adult life to maintain femininity, sexual organs and bone health, and enhance gender and sexuality. Among males, appropriate testosterone therapy maintains stamina, muscle tone, bone health, libido, sexual potency and general well-being, while benefit for healing after genital surgery is unclear. Further, outcome is clearly related to predominant cultural factors. Outcome studies should include evaluation of all of these factors.

Original languageEnglish (US)
Pages (from-to)611-615
Number of pages5
JournalJournal of Pediatric Urology
Volume8
Issue number6
DOIs
StatePublished - Dec 1 2012

Fingerprint

Disorders of Sex Development
Psychology
Therapeutics
Outcome Assessment (Health Care)
Femininity
Patient Advocacy
Social Adjustment
Bone and Bones
Libido
Congenital Adrenal Hyperplasia
Hypospadias
Cryptorchidism
Aptitude
Coitus
Reproductive Health
Disclosure
Sexuality
Puberty
Cosmetics
Personality

Keywords

  • Disorders of sexual development (DSD)
  • Genital surgery
  • Gonadal hormone therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Urology

Cite this

Review of recent outcome data of disorders of sex development (DSD) : Emphasis on surgical and sexual outcomes. / Lee, Peter; Schober, Justine; Nordenström, Anna; Hoebeke, Piet; Houk, Christopher P.; Looijenga, Leendert; Manzoni, Gianantonio; Reiner, William; Woodhouse, Christopher.

In: Journal of Pediatric Urology, Vol. 8, No. 6, 01.12.2012, p. 611-615.

Research output: Contribution to journalArticle

Lee, P, Schober, J, Nordenström, A, Hoebeke, P, Houk, CP, Looijenga, L, Manzoni, G, Reiner, W & Woodhouse, C 2012, 'Review of recent outcome data of disorders of sex development (DSD): Emphasis on surgical and sexual outcomes', Journal of Pediatric Urology, vol. 8, no. 6, pp. 611-615. https://doi.org/10.1016/j.jpurol.2012.10.017
Lee, Peter ; Schober, Justine ; Nordenström, Anna ; Hoebeke, Piet ; Houk, Christopher P. ; Looijenga, Leendert ; Manzoni, Gianantonio ; Reiner, William ; Woodhouse, Christopher. / Review of recent outcome data of disorders of sex development (DSD) : Emphasis on surgical and sexual outcomes. In: Journal of Pediatric Urology. 2012 ; Vol. 8, No. 6. pp. 611-615.
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