[Clinical, cytogenetical, histological, immunological and hormonal studies in a case of true hermaphroditism (author's transl)].
A true hermaphrodite with ambiguous genitalia and 46 XX caryotype was investigated during adolescence. At 13 years testosterone concentrations (ng/ml) were 6 and 390 respectively in peripheral and right ovotestis venous blood. After removal of the right gonad, large fluctuations of estradiol levels (40 to 220 pg/ml) were observed. But the testosterone secretion by the left ovotestis was low, although responsive to hCG. A significant LH surge was induced by ethinyl-estradiol load before removal of the left gonad. The 5 alpha-reductase activity was normal in the pubic skin and the levels of cytosolic receptors of testosterone and DHT were high in the left gonad. The presence of H-Y antigen was demonstrated on lymphocytes.
D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase / Q000378:metabolism; D000293:Adolescent; D002648:Child; D012734:Disorders of Sex Development / Q000276:immunology / Q000378:metabolism*; D005260:Female; D012739:Gonadal Steroid Hormones / Q000097:blood; D006182:H-Y Antigen / Q000032:analysis; D006801:Humans; D011944:Receptors, Androgen / Q000032:analysis
D012739:Gonadal Steroid Hormones; D006182:H-Y Antigen; D011944:Receptors, Androgen; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase
Etudes cliniques, cytogénétiques, histologiques, immunologiques et hormonales chez un hermaphrodite.