Long term outcomes in 46, XX adult patients with congenital adrenal hyperplasia reared as males

Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years’ Experience and Challenges

“…For the determinative factors to recommend gender assignment in 46,XX cases; besides the etiological diagnosis, age, psychologic gender and Prader staging were showed us how important for assignment decisions (Table 2,4). Similar to our study, Khattab et al (13) report three 46,XX with 21-OHD CAH patients who reared as male gender. In another study, of the 50 DSD patients, the 4/11 cases diagnosed with 46,XX DSD due to CAH had assumed male gender role (15).…”

“…And this condition is the main structure of sexual identity, and it is influenced by genetic status, pre/postnatal exposure to androgens, sociocultural factors, and family dynamics (6,39,48,49). Gender assignment is an important problem in DSD patients who have virilized brain with undervirilized external genitalia (13)(14)(15)39). Eleven of 97 46,XX patients (11.3%) had male gender orientation in the psychological evaluation, and raised as male gender by parents (9 were 21-OHD, 1 was 11-OHD, and 1 was Sertoli cell only syndrome, mean age of cases was 9.92 ± 4.96 years).…”

“…The long-term physical, social and psychological outcomes of patients with DSD are still unclear. There are increasing concerns regarding early decisions about gender assignment in recent reports (13)(14)(15)(16)(17)(18). Studies were generally conducted on psychosexual and surgical outcomes of this group of patients (19)(20)(21)(22).…”

Bilateral Adrenal Myelolipomas Presenting as Acute Adrenal Insufficiency in an Adult With Congenital Adrenal Hyperplasia

“…They were later self-reassigned to male gender due to dissatisfaction of living as females. A case series by Khattab et al reported three 46,XX CAH patients 15 . Two patients, who were assigned to male gender and underwent surgical removal of female reproductive organs and male genitoplasty, showed satisfaction in regards to male gender role and behavior.…”

Hetero sexual precocious puberty: Case report and literature review

“…However in the study by Khattab A etal three patients of CAH with 46 XX karyotype were assigned male gender and had a successful outcome provided there is strong parental support and expert endocrine care. [13] Clinical criteria for diagnosis An infant presenting with salt wasting crisis in the first four weeks of life must be suspected of CAH. These infants can have raised serum concentration of potassium, low serum concentration of sodium, chloride, and total carbon dioxide (CO 2 ) and inappropriately raised urine concentration of sodium.…”