Sexual Development Disorder Revealing 11-Beta-Hydroxylase Deficiency
Journal Title: Scholars Journal of Applied Medical Sciences - Year 2018, Vol 6, Issue 7
Abstract
Congenital adrenal hyperplasia (CAH) by 11-Beta-hydroxylase deficiency is a rare autosomal recessive disorder that accounts for 5-8% of CAH. We report the observation of one CAH discovered during a sexual development disorder with severe hypertension resistant to treatment in a girl aged 15 years, raised as a girl. A feminization surgery was proposed, a feminizing genitoplasty was performed without adrenalectomy because of the correction of blood pressure figures under medical treatment. Late diagnosis of CAH poses a problem of sexual orientation, so antenatal diagnosis and treatment are essential to avoid virilization of the female fetus.
Authors and Affiliations
N. Belhamri, G. El Mghari, N. Al Ansari
Keywords
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