Abstract:
:Six Arabs subjects (three postpubertal, two prepubertal and one pubertal) from three interrelated Omani families with male pseudohermaphroditism due to 5 alpha-reductase-2 deficiency were evaluated. These subjects had been raised as girls since birth as they were born with a clitoral-like phallus and ambiguous external genitalia of pseudovaginal perineoscrotal hypospadias with separate urethral and vaginal orifices. They underwent variable degrees of increased muscular habitus and phallic enlargement during puberty and beyond. Gynaecomastia was absent and the body and facial hair was insignificant. After diagnosis, a transition to male social sex occurred in two cases, one of which was interventional. Two retained the female social sex, one of which was also interventional, while the other two maintained an equivocal gender status. This report provides new data on the characterisation of 5 alpha-reductase-2 deficiency in various clusters.
journal_name
Postgrad Med Jjournal_title
Postgraduate medical journalauthors
al-Attia HMdoi
10.1136/pgmj.73.866.802subject
Has Abstractpub_date
1997-12-01 00:00:00pages
802-7issue
866eissn
0032-5473issn
1469-0756journal_volume
73pub_type
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