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J Pediatr Endocrinol Metab ; 26(9-10): 915-20, 2013.
Article in English | MEDLINE | ID: mdl-23729553

ABSTRACT

There are few reports of adults with disorders of sexual development (DSD). Here we describe the clinical profile and results of psychological assessment of three siblings with 46, XY DSD caused by partial androgen insensitivity syndrome (PAIS). The elder sibling (aged 22 years) was reared as female, while the middle and youngest siblings (17 and 18 years of age), were reared as males. The gender identity was concordant with the sex of rearing. There was no gender dysphoria. The psychological distress that our patients experienced was due to the limitations placed on them by their medical condition. It did not permit them to experience various facets of being either male or female completely. The younger siblings reared as males had additional problems of gynecomastia and lack of male secondary sexual development.


Subject(s)
Adolescent Development , Androgen-Insensitivity Syndrome/physiopathology , Cost of Illness , Gender Identity , Psychosexual Development , Stress, Psychological/etiology , Adolescent , Adult , Androgen-Insensitivity Syndrome/diagnosis , Androgen-Insensitivity Syndrome/psychology , Androgen-Insensitivity Syndrome/therapy , Delayed Diagnosis , Female , Humans , Male , Pedigree , Self Concept , Siblings , Treatment Outcome , Young Adult
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