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[Clinical, cytogenetical, histological, immunological and hormonal studies in a case of true hermaphroditism (author's transl)].

M Roger, Christopher D. Roy, M Fellous, L Boccon-Gibod, Jorge Raul Pasqualini, R Bonno, Jean Paul Harpey

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Abstract

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.

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What this paper is about

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.

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Available abstract

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.

Key concepts: Ovotestis, Gonad, True hermaphroditism, Testosterone (patch), Internal medicine, Endocrinology, Biology, Hermaphrodite

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[Clinical, cytogenetical, histological, immunological and hormonal studies in a case of true hermaphroditism (author's transl)]. — Research Paper | ScholarLens