DANAZOL SUPPRESION OF GONADAL ESTRADIOL 17β SECRETION : REPORT OF TWO SIBLING CASES WITH A COMPLETE FORM OF TESTICULAR FEMINIZATION ON GONADAL ULTRASTRUCTURE AND ENDOCRINE STATUS

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It has been established that the pathogenesis of a complete form of testicular feminization syndrome (TFM) is due to end organ unresponsiveness to androgens. In the present study, two sibling cases with TFM have been reported with special reference to the effect of danazol (a derivative of 17α-ethynyltestosterone) on gonadal estradiol 17β (E_2) secretion in vivo and in vitro. Both patients showed hypergonadotropic-hypergonadism with good response to LH RH. In one case, peripheral blood E_2 levels seemed to fall independently of gonadotropins during danazol administration in vivo. Administrations of danazol to the culture media also inhibited E_2 Secretion from the cultured monolayered cells of the testes. These results suggest a direct inhibitory effect of danazol on gonadal E_2 secretion. However, the feedback mechanisms of hypothalamic pituitary units to E_2 were preserved in both cases. Spermatogeneses were almost undetectable as in immature embryonal testes when using either light or electron microscopies, though spermatogonia were differentiated from Sertoli cells by these techniques. Finally, plasma T and E_2 levels in both patients were considerably lower after gonadectomy
社団法人日本産科婦人科学会の論文
1984-01-01