Very late presentation of a disorder of sex development

Martins, J.M.; Fraga, M.; Miguens, J.; Tortosa, F.; Marques, B.; Sousa, A.D.

http://hdl.handle.net/10400.18/4831

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Abstract(s)

Disorders of sex development generally present in the neonatal period with ambiguity of external genitalia. We report a very old male patient presenting at 75 years because of panhypopituitarism and a large nonsecreting pituitary macroadenoma secondary to long-standing primary hypogonadism due to 46,XX sex reversal disorder now first diagnosed. Sex development disorders may go unrecognised for the entire life span, despite infertility and long-standing primary gonadic failure may lead to uncommon complications.