A case of complete testicular feminization syndrome with Inguinal Hernias / 대한산부인과학회잡지

ABSTRACT

Complete testicular feminization syndrome (androgen insensitivity syndrome) is the most common form of male pseudohermaphroditism, transmitted by means of maternal X-linked recessive gene. This syndrome is marked by unique combination; Normal female phenotype, normal male karyotype, 46,XY, normal or slightly elevated male blood testosterone levels and a high LH. Clinically, the diagnosis should be considered in a female with inguinal hernias, a patient with primary amenorrhea and absent uterus. we present a case testicular feminization syndrome with blindly ended vagina and inguinal masses with a brief review of literatures.