Global Disorders of Sex Development Update since 2006: Perceptions, Approach and Care

Abstract

The goal of this update regarding the diagnosis and care of persons with disorders of sex development (DSDs) is to address changes in the clinical approach since the 2005 Consensus Conference, since knowledge and viewpoints change. An effort was made to include representatives from a broad perspective including support and advocacy groups. The goal of patient care is focused upon the best possible quality of life (QoL). The field of DSD is continuously developing. An update on the clinical evaluation of infants and older individuals with ambiguous genitalia including perceptions regarding male or female assignment is discussed. Topics include biochemical and genetic assessment, the risk of germ cell tumor development, approaches to psychosocial and psychosexual well-being and an update on support groups. Open and on-going communication with patients and parents must involve full disclosure, with the recognition that, while DSD conditions are life-long, enhancement of the best possible outcome improves QoL. The evolution of diagnosis and care continues, while it is still impossible to predict gender development in an individual case with certainty. Such decisions and decisions regarding surgery during infancy that alters external genital anatomy or removes germ cells continue to carry risk.

The human rights of intersex people: addressing harmful practices and rhetoric of change

“…A 2016 follow-up statement does the same. 30 The authoritativeness and "consensus" in the Chicago statement lies not in comprehensive clinician input or meaningful community input, but in its utility to justify any and all forms of clinical intervention. Clinical practices have not been impacted by the statement; they have been validated.…”

Gender identity, gender assignment and reassignment in individuals with disorders of sex development: a major of dilemma

“…Individual DSD outcome data regarding gender identity, quality of life (QoL), avoidance of unnecessary surgery, hormone replacement, and fertility preservation must be considered in deciding gender assignment [72][73][74]. Sexual potential, one the most difficult aspect to predict, is also a key factor to be considered for male or female assignment [75]. Particularly, penis length and its potential to develop during puberty into a sexually functional penis are crucial aspects to take into account if male assignment is considered.…”

“…Despite the majority of individuals with 5-alpha-reductase-2 deficiency present a male gender identity, it should be considered that those assigned female have been reported with satisfactory sexual activity [77]. Among patients with partial androgen insensitivity syndrome (PAIS), male gender assignment should be considered when there is a satisfactory response to testosterone therapy in terms of phallic growth, as well as an established causative genetic variant [75]. A female assignment is suggested if testosterone treatment is without effects [75].…”

“…Among patients with partial androgen insensitivity syndrome (PAIS), male gender assignment should be considered when there is a satisfactory response to testosterone therapy in terms of phallic growth, as well as an established causative genetic variant [75]. A female assignment is suggested if testosterone treatment is without effects [75]. Regarding persons with 17-beta-hydroxysteroid dehydrogenase type 3 (17-beta-HSD-3) deficiency, sexual dissatisfaction has been reported in both male and female gender assignments [75].…”

“…A female assignment is suggested if testosterone treatment is without effects [75]. Regarding persons with 17-beta-hydroxysteroid dehydrogenase type 3 (17-beta-HSD-3) deficiency, sexual dissatisfaction has been reported in both male and female gender assignments [75].…”