Abstract
3 patients with late-onset 3 beta-HSD deficiency are described. They presented increased DHEAs/delta 4 ratio either in basal conditions or after ACTH stimulation. All patients were hirsute but only one had increased serum values of delta 4 and total testosterone. However, all patients presented increased serum levels of free testosterone. Two patients had normal menstrual cycles and in one case a secondary PCO was found.
MeSH terms
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3-Hydroxysteroid Dehydrogenases / deficiency*
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Adolescent
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Adrenocorticotropic Hormone
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Dehydroepiandrosterone / analogs & derivatives
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Dehydroepiandrosterone / blood
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Dehydroepiandrosterone Sulfate
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Female
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Hirsutism / enzymology
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Humans
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Menstrual Cycle
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Polycystic Ovary Syndrome / complications
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Sex Hormone-Binding Globulin / analysis
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Testosterone / blood
Substances
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Sex Hormone-Binding Globulin
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Testosterone
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Dehydroepiandrosterone
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Dehydroepiandrosterone Sulfate
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Adrenocorticotropic Hormone
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3-Hydroxysteroid Dehydrogenases