Affordable Access

Publisher Website

Disorders of sex development: a new definition and classification

Authors
Journal
Best Practice & Research Clinical Endocrinology & Metabolism
1521-690X
Publisher
Elsevier
Publication Date
Volume
22
Issue
1
Identifiers
DOI: 10.1016/j.beem.2007.11.001
Keywords
  • Ambiguous Genitalia
  • Karyotype
  • Consensus
  • Nomenclature
Disciplines
  • Communication

Abstract

A newborn infant with ambiguous genitalia is a complex enough problem to unravel without any further clouding by confusing terms. The nomenclature ‘intersex’, ‘hermaphrodite’ and ‘pseudohermaphrodite’ is anachronistic, unhelpful, and perceived to be pejorative by some affected families. In its place, a consensus statement recommends the term ‘disorder of sex development’ (DSD), a generic definition encompassing any problem noted at birth where the genitalia are atypical in relation to the chromosomes or gonads. The karyotype is used as a prefix to define the category of DSD, replacing the arcane terminology of male or female pseudohermaphroditism (now known as XY DSD or XX DSD, respectively). The new nomenclature has spawned a simple and logical classification of the causes of DSD. In this chapter new facets of gonadal dysgenesis and novel defects in steroid biosynthesis are reviewed in relation to the DSD classification, and options for early, non-invasive fetal sexing are described. Future research to determine many causes of DSD will benefit from the use of this universal language of scientific communication.

There are no comments yet on this publication. Be the first to share your thoughts.

Statistics

Seen <100 times
0 Comments

More articles like this

Definition and classification of female sexual dis...

on International journal of impot... May 1998

[New classification of sex disorders].

on Ginekologia Polska August 1980

Disorders of sex development: new genes, new conce...

on Nature Reviews Endocrinology February 2013

Clinical Review of 91 Patients with 46, XX Disorde...

on Journal of Pediatric and Adole...
More articles like this..