Published online August 1, 2006
PEDIATRICS Vol. 118 No. 2 August 2006, pp. 753-757 (doi:10.1542/peds.2006-0737)
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SPECIAL ARTICLE

Summary of Consensus Statement on Intersex Disorders and Their Management

Christopher P. Houk, MD, PhDAa, Ieuan A. Hughes, FMedSci, FRCPCHb,c, S. Faisal Ahmed, FRCPCHd, Peter A. Lee, MD, PhDe,c and Writing Committee for the International Intersex Consensus Conference Participants

a Department of Pediatrics, Backus Children’s Hospital, Mercer University School of Medicine, Savannah, Georgia
b Department of Paediatrics, University of Cambridge, Addenbrooke’s Hospital, Cambridge, United Kingdom
c Co-chairs
d Department of Child Health, Royal Hospital for Sick Children, Glasgow, Scotland, United Kingdom
e Department of Pediatrics, Penn State College of Medicine, Milton S. Hershey Medical Center, Hershey, Pennsylvania

Key Words: intersex • sexual differentiation • ambiguous genitalia • genital surgery

Abbreviations: DSD—disorder(s) of sex development • AE—androgen exposure • CAH—congenital adrenal hyperplasia • 5{alpha}-RD2—5{alpha}-reductase deficiency • CAIS—complete androgen insensitivity syndrome

The first 300 words of the full text of this article appear below.

Advances in understanding of genetic control of sexual determination and differentiation, improvements in diagnostic testing and surgical genital repair, and the persistent controversies inherent to clinical management were all compelling factors that led to the organization of an international consensus conference. The goals were to acknowledge and discuss the more controversial issues in intersex management, provide management guidelines for intersex patients, and identify and prioritize questions that need additional investigation. This is a summary statement.


   NOMENCLATURE AND DEFINITIONS
 
Advances in molecular genetic causes of abnormal sexual development and heightened awareness of the ethical and patient-advocacy issues mandate reexamination of existing nomenclature for patients with intersex.1 Terminology such as "pseudohermaphroditism" is controversial, potentially pejorative to patients,2 and inherently confusing. Therefore, the term "disorders of sex development" (DSD) is proposed to indicate congenital conditions with atypical development of chromosomal, gonadal, or anatomic sex.

Additional rationale for new classification is the need for modern categorization to integrate the modern molecular genetic aspects, to maximize precision when applying definitions and diagnostic labels,3 and to meet the need for psychologically sensitive yet descriptive medical terminology. Nomenclature should be flexible enough to incorporate new information, robust enough to maintain a consistent framework, use descriptive terms, reflect genetic etiology, accommodate phenotypic variation spectrum, and be useful for clinicians, scientists, patients, and families. Hence, we propose a new classification (see "Consensus Statement on Management of Intersex Disorders"4 in this month's issue of Pediatrics Electronic Edition).

Three traditionally conceptualized domains of psychosexual development are gender identity (one's self-representation [ie, male or female]), gender role (sexually dimorphic behaviors within the general population, such as toy preferences, aggression, and spatial ability), and sexual orientation (direction[s] of erotic interest). Gender dissatisfaction denotes unhappiness with assigned sex and may result in gender self-reassignment. Psychosexual developmental factors relate to parental psychopathology, parent-child attachment, or parenting practices . . . [Full Text of this Article]

Address correspondence to Peter A. Lee, MD, PhD, Department of Pediatrics, MC-H085, Penn State College of Medicine, Milton S. Hershey Medical Center, Box 850, 500 University Dr, Hershey, PA 17033-0850. E-mail: plee@psu.edu




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