وثيقة

Male pseudohermaphroditism due to 5α-reductase-2 deficiency in an Arab kindred.

المعرف
DOI: 10.1136/pgmj.73.866.802
المصدر
Postgraduate Medical Journal. v. 73, 866, p. 802-807
الدولة
United Kingdom.
مكان النشر
London
الناشر
BMJ Publishing Group.
ميلادي
1997-12-01
اللغة
الأنجليزية
الموضوع
الملخص الإنجليزي
Six Arabs subjects (three postpubertal, two prepubertal and one pubertal) from three interrelated Omani families with male pseudohermaphroditism due to 5α-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 5α-reductase-2 deficiency in various clusters.
ISSN
0032-5473
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