Abstract
Congenital adrenal hyperplasia (CAH) is a genetic condition of the steroidogenic enzymes in the adrenal cortex normally leading to variable degrees of cortisol and aldosterone deficiency as well as androgen excess. Exposure to androgens prenatally might lead to ambiguous genitalia. The fetal brain develops in traditional male direction through a direct action of androgens on the developing nerve cells, or in the traditional female direction in the absence of androgens. This may indicate that sexual development, including sexual orientation, are programmed into our brain structures prenatally. The objective of this study was to perform a systematic review of the literature, investigating sexual orientation in individuals with CAH. The study also aimed at identifying which measures are used to define sexual orientation across studies. The review is based on articles identified through a comprehensive search of the OVIDMedline, PsycINFO, CINAHL, and Web of Science databases published up to May 2019. All peer-reviewed articles investigating sexual orientation in people with CAH were included. Quantitative, qualitative, and mixed methods were considered, as well as self-, parent-, and third-party reports, and no age or language restrictions were enforced on publications. The present review included 30 studies investigating sexual orientation in patients with CAH assigned female at birth (46, XX) (n = 927) or assigned male at birth (46, XY and 46, XX) (n = 274). Results indicate that assigned females at birth (46, XX) with CAH had a greater likelihood to not have an exclusively heterosexual orientation than females from the general population, whereas no assigned males at birth (46, XY or 46, XX) with CAH identified themselves as non-heterosexual. There was a wide diversity in measures used and a preference for unvalidated and self-constructed interviews. Hence, the results need to be interpreted with caution. Methodological weaknesses might have led to non-heterosexual orientation being overestimated or underestimated. The methodological challenges identified by this review should be further investigated in future studies.
Highlights
The first known patient with congenital adrenal hyperplasia (CAH) was described in 1865 by Luigi De Crecchio, a professor of anatomy in Napoli (Delle Piane et al, 2015)
We explored which dimensions of sexual orientation that had been measured in the different studies, widening the scope of the review
The current review indicates that females with CAH more often than the general population define themselves as nonheterosexual, which could have clinical implications
Summary
The first known patient with congenital adrenal hyperplasia (CAH) was described in 1865 by Luigi De Crecchio, a professor of anatomy in Napoli (Delle Piane et al, 2015). The patient had a six centimeter long penis but no testes. At birth, he was regarded a female, but at 4 years of age, he was reconsidered a male. He was regarded a female, but at 4 years of age, he was reconsidered a male As an adult, he fell in love with a girl; when he proposed to marry her, she ran away after realizing he was called Giuseppina in his birth certificate (Delle Piane et al, 2015). CAH is a common cause of disorders of sex development (DSD), and Guiseppe’s story is still relevant today
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