In 2006 a panel of professionals, parents and affected individuals collaborated to generate a consensus statement on management of individuals with intersex conditions. (see Reference 9 below) New nomenclature was introduced with the goal of minimizing pejorative terms. This has become a common and specific language for the medical community. Disorders of Sex Development (DSD) (also called Disorder of Sex Differentiation), was preferred over intersexuality and the prior hermaphroditism. (see Table 1). A review of 60 European DSD centers showed that a majority implemented policies and procedures in accordance with the recommendations issued by the 2006 Consensus Group, representing a change in practice with the collaborative goal of improved patient care.1 More recently there is dissatisfaction within the affected community with nomenclature that suggests a disorder. Some affected congenital adrenal hyperplasia (CAH) patients/parents prefer to separate CAH from DSD and a small sample study of the Androgen Insensitivity Syndrome–DSD Support Group showed that affected parents have negative views about the DSD terminology commonly used by medical professionals.2 While there is no clear consensus, individuals may prefer use of DSD, difference of sex development, intersex or a specific diagnosis and re-evaluation of current nomenclature, in collaboration with advocates, is needed.
1.1 Key words
Disorder of sex development, difference of sex development, dysgenesis, gender, gender identity, genital ambiguity, gonad, intersex, ovotestis, sex of rearing
Regardless of the preferred terminology, this collection of diagnoses involve discordance among three processes: chromosomal, gonadal, or phenotypic sex determination. Human sex development occurs in an organized, sequential manner.3,4,5,6,7,8,9,10,11,12,13,14,15,16,17 Chromosomal sex is established at fertilization, which then directs the undifferentiated gonads to develop into testes or ovaries. Phenotypic sex results from the differentiation of internal ducts and external genitalia under the influence of hormones and transcription factors. Sexual differentiation is regulated by more than 50 different genes on both the sex chromosomes and autosomes.7,8,15,16
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