A Case Report of Complete Testicular Feminization Syndrome with Rudimentary Salpinx / 대한산부인과학회잡지

ABSTRACT

The Complete testicular feminization syndrome is a hereditary syndrome characterized clinically by female phenotype with 46, XY karyotype and bilateral testes. There is a congenital insensitivity to androgens, transmitted by means of a maternal X-linked recessive gene responsible for the androgen intracellular receptor. Therefore, androgen induction of Wolffian duct development does not occur. However, anti-mullerian hormone activity is present and the individual does not have mullerian development. Principle of treatment is reinforced to live normal female life. This is a case report of testicular feminization syndrome with rudimentary salpinx with the brief review of literatures.