Gonadotropin release in patients with androgen insensitivity. Testicular feminization syndrome

Two siblings are presented, both with classic androgen-insensitivity syndrome (AIS). Endocrine testing was carried out and then gonadectomy was performed. Studies in these patients with AIS revealed low control serum FSH. In these patients serum FSH was not significantly suppressed by intravenous estrogen nor adequately stimulated by FSH-LH-releasing hormone but rose normally after castration. The various studies are interpreted in the light of the known kinetics of pituitary gonadotorpins and their modulation by gonadal steroids. A third sibling is also presented.