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Korean Journal of Obstetrics & Gynecology 1997;40(4):896-901.
Published online January 1, 2001.
A case of male Pseudohermaphroditism due to 17alpha-Hydroxylase Deficiency.
Jae Hoon Kim, So Young Choi, Jang Heub Kim, Jin Hong Kim, Jong Gu Rha, Chang Suk Kang
1Department of Obsterics and Gynecology, Catholic University Medical College, Korea.
2Department of clinical pathology, Catholic University Medical College, Korea.
Abstract
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.
Key Words: 17alpha-hydroxylase, Male pseudohermaphroditism, Congenital adrenal hyperplasia


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