A 21-year-old phenotypic female with 46,XY genotype presented with primary amenorrhea, headache, absence of secondary sex characteristics, and hypertension.Further evaluation confirmed male pseudohermaphroditism having no nterus and adnexase.After basic hormonal study and ACTH stimulation test, we concluded 17alpha-hydroxylase deficiency. Owing to the high risk of gonadal neoplasia in XY gonadal streaks, prophylactic removal of the steaks is recommended. Traditionally this procedure has been performed by laparotomy, but in this case laparoscopic gonadctomy was performed. Following treatment with hydrocrtisone, potassium, progesterone, 11-deoxycorticosterone, corticosterone and urinay pregnanediol levels were normalized. normal blood pressure measurements were achieved during treatment with hydrocortisone and estrogen with the patient. We report this case with a breif review of the literatures.
46, XY Disorders of Sex Development* ; Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Amenorrhea ; Blood Pressure ; Corticosterone ; Estrogens ; Female ; Genotype ; Gonads ; Headache ; Humans ; Hydrocortisone ; Laparotomy ; Male* ; Potassium ; Pregnanediol ; Progesterone ; Sex Characteristics ; Young Adult