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      Dexamethasone-Suppressible Hypercorticosteronism in Two 46, XX Subjects with Ambiguous Genitalia and Ovarian Cysts

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          The paradoxical association of female pseudohermaphroditism and androgen deficiency was observed in two 46, XX subjects with high corticosterone plasma levels. Subject 1 has been declared a boy due to clitoris enlargement; she had no vagina and uterus. Subject 2 had ambiguous external genitalia. In both, at age 27 and 17 years, fusion of outer labia, impuberism, ovarian cysts, and histologically normal ovarian tissue were observed. Blood pressure was normal. Basal cortisol levels were normal but unresponsive to ACTH. Progesterone levels were 40 and 62 ng/ml and rose after ACTH (50 and 79 ng/ml). 17-hydroxyprogesterone levels were 25 and 21 ng/ml and did not rise after ACTH. Corticosterone levels were 70 and 92 ng/ml and rose after ACTH (110 and 180 ng/ml). All three steroids were suppressed by dexamethasone. Androgen and estrogen levels were at or below the lower limit for normal women. The sex steroid levels obtained by radioimmunoassay in plasma and a follicular cyst fluid were confirmed by isotope dilution-mass spectrometry. We suggest that the sexual ambiguousness resulted from an excessive production of gestagenic steroids during fetal life, and that the enzyme defect is either a partial 17α-hydroxylase defect combined with a peripheral production of 17-hydroxyprogesterone, or else a partial 17–20-desmolase defect with a secondary 21-hydroxylase defect limited to the cortisol pathway.

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          Author and article information

          Horm Res Paediatr
          Hormone Research in Paediatrics
          S. Karger AG
          25 November 2008
          : 16
          : 1
          : 23-31
          aFondation de Recherche en Hormonologie, Paris et Fresnes, France; bHôpital Bichat, cHôpital Trousseau et dHôpital Saint-Vincent-de-Paul, Paris, France; eHôpital Beaujon, Clichy, France
          179481 Horm Res 1982;16:23–31
          © 1982 S. Karger AG, Basel

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          Page count
          Pages: 9


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