hrp0094p2-419 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

A late revelation of Sexual differentiation disorder 46XX due to 11 beta hydroxylase deficiency

Sabrina Khensal , Amina Bouhlassa , Chahinez Habi , Nassim Nouri

Introduction: Congenital adrenal hyperplasia due to 11 beta hydroxylase deficiency, is an autosomal recessive pathology that represents 5 to 8% of congenital adrenal hyperplasia. The predominant symptoms of this disorder are androgen excess’ features, including masculinization of female newborns and precocious puberty in male children. Approximately, two thirds of patients also have hypertension, which may or may not be associated with mineralocorticoid excess, hypokale...