[Female pseudohermaphroditism].
Contrary to what is observed in true hermaphroditism and in male pseudo-hermaphroditism, there is no erroneous transmission of the genetic gonadal differentiation programme in female pseudohermaphroditism. All that has happened is virilization of the urogenital sinus and external genitalia in a foetus exposed to exo- or endogenous androgens. In the absence of testis there is no production of anti-mullerian hormone, and for these reasons the uterus, the fallopian tubes and the vagina develop normally, whilst the wollfian ducts are regressed and there is no trace of male deep spermatic pathway. On the basis of recent acquisitions in biochemistry, endocrinology and embryogenesis, the authors compare the various degrees of virilization with the biochemical abnormalities.
Minh
H N
HN
Service central d'Anatomie et de Cytologie pathologiques, CHRU, Amiens.
Belaisch
J
J
Smadja
A
A
D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase / Q000502:physiology; D000312:Adrenal Hyperplasia, Congenital / Q000378:metabolism; D013196:Dihydrotestosterone / Q000494:pharmacology; D012734:Disorders of Sex Development / Q000196:embryology* / Q000378:metabolism / Q000473:pathology; D005314:Embryonic and Fetal Development; D005260:Female; D005836:Genitalia, Female / Q000196:embryology* / Q000473:pathology; D005837:Genitalia, Male / Q000196:embryology* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012733:Sex Differentiation; D013250:Steroid Hydroxylases / Q000172:deficiency
D013196:Dihydrotestosterone; D015096:3-Hydroxysteroid Dehydrogenases; D013250:Steroid Hydroxylases; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase
Pseudo-hermaphrodisme féminin.