Gender identity and role in a pedigree of Arabs with intersex due to 5 alpha reductase-2 deficiency.
Between 1986 and 1995, a pedigree of six Arabs with male pseudohermaphroditism due to 5 alpha reductase-2 deficiency have been identified. All, were raised as girls since birth. At the time of diagnosis, three were post-pubertal, one pubertal and two pre-pubertal. The external genitalia of 'pseudo-vaginal perineoscrotal hypospadias' was identical in these subjects. Although these individuals were a homogeneous group in terms of their sex of upbringing, phenotypic appearance, endocrinological profile and socio-cultural background, the development of the gender identity and role was not uniform in these six cases. Their psycho-sexual make-up was closely related to the transaction of their life experiences. These cases provide further insight into the interaction between various factors involved in the development of gender identity and role in male pseudohermaphrodites in an Eastern culture.
al-Attia
H M
HM
Department of Internal Medicine, Mafraq Hospital Abu Dhabi, United Arab Emirates.
D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase / Q000172:deficiency* / Q000235:genetics; D000293:Adolescent; D000328:Adult; D018912:Arabs / Q000235:genetics* / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D003466:Cultural Characteristics; D012734:Disorders of Sex Development / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D005260:Female; D006801:Humans; D007527:Isoenzymes / Q000172:deficiency* / Q000235:genetics; D008297:Male; D010375:Pedigree; D010641:Phenotype; D011606:Psychosexual Development; D012951:Socialization
D007527:Isoenzymes; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase