[Incomplete testicular feminisation syndrome by testosterone insensitivity (author's transl)].
Male pseudo-hermaphroditism by partial testosterone sensitivity, without steroido-genesis abnormality, without apparent 5 alpha reductase deficit - Empty complete scrotum and hypospadias surgically normalized. At 39, scrotal testis 32 X 15 mm right, 23 X 10 mm left. Infantile penis and prostata. Gynoid scapulo-pelvic proportions, adipo-muscular ratio and fat distribution. Bilateral gynecomastia. Caryotype 46 XY - 17 ks 26 mg. After intravenous 100 ng LH RH: plasma (pl) LH increased 105 to 285 ng/ml, pl testosterone 5 ng/ml unchanged, pl oestradiol 115 to 135 pg/ml. 48 h after intra muscular 300 mg testosterone propionate: pl testosterone increased, 13 to 39 ng/ml, androstenedione 1,5 to 3,04 ng/ml, dihydrotestosterone 0,85 to 1,45 ng/ml, OEstradiol 52 to 110 pg/ml. No effect of 29 g testosterone oenanthate in 2 years.
D000328:Adult; D012734:Disorders of Sex Development / Q000503:physiopathology*; D007987:Gonadotropin-Releasing Hormone; D006801:Humans; D007621:Karyotyping; D008297:Male; D010413:Penis / Q000033:anatomy & histology; D011467:Prostate / Q000033:anatomy & histology; D013737:Testis / Q000033:anatomy & histology; D013739:Testosterone
D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone
Pseudo-hermaphrodisme mâle incomplet par insensibilité à la testostérone