46,XX patients with congenital adrenal hyperplasia: initial assignment as male, reassigned female.
Six 46,XX patients with congenital adrenal hyperplasia (CAH) presented with genital ambiguity, five so severe that initial gender assignment was male. Once diagnosis was realized, parents were involved in evaluation and chose sex re-assignment as female. To date, these girls and their parents all indicate satisfaction with their decision for a female sex of rearing. The girls have a female gender identity with behavior characteristics known for females with CAH. Thus, while outcome is satisfactory, it is realized that for most, expression of sexual orientation and adult life adjustments have not yet occurred.
Lee
Peter A
PA
Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. plee@psu.edu
Witchel
Selma F
SF
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000523:psychology / Q000601:surgery; D002648:Child; D002652:Child Behavior; D002657:Child Development; D041321:Chromosomes, Human, X; D012734:Disorders of Sex Development / Q000209:etiology / Q000523:psychology / Q000601:surgery; D005260:Female; D005783:Gender Identity; D023961:Gonadal Dysgenesis, 46,XX / Q000150:complications* / Q000523:psychology / Q000601:surgery; D006801:Humans; D007062:Identification, Psychological; D007231:Infant, Newborn; D009034:Mother-Child Relations; D011606:Psychosexual Development; D014770:Virilism / Q000209:etiology / Q000523:psychology* / Q000601:surgery