Journal of Clinical Endocrinology & Metabolism: “Approach to assigning gender in 46,XX congenital adrenal hyperplasia with male external genitalia: Replacing dogmatism with pragmatism”

The goal of sex assignment is to facilitate the best possible quality of life for the patient. Factors such as reproductive system development, sexual identity, sexual function, and fertility are important considerations in this regard. Although some DSD gender assignments are relatively straightforward, those with midstage genital ambiguity and unclear gonadal function represent a major challenge.

The Journal of Clinical Endocrinology & Metabolism: Approach to Assigning Gender in 46,XX Congenital Adrenal Hyperplasia with Male External Genitalia: Replacing Dogmatism with Pragmatism

A recent major change in DSD care has been to encourage a male assignment for 46,XY infants with ambiguous genitalia who have evidence of testicular function and in utero central nervous system androgen exposure. In contrast, assignment of virilized 46, XX DSD patients remains female when ovaries and internal organs are present, regardless of the extent of virilization of the external genitalia. In this paper, we propose consideration of male assignment for these 46,XX patients who have fully developed male genitalia based on available outcome data.

The medical profession has woken up to the fact that nobody can predict the result when attempts are made to feminize the more extreme manifestations of CAH. However – the same thing also applies in reverse.

We call for a patient-centred approach based on free, fully informed and prior consent.

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