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

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          Abstract

          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

          Journal
          HRE
          Horm Res Paediatr
          10.1159/issn.1663-2818
          Hormone Research in Paediatrics
          S. Karger AG
          1663-2818
          1663-2826
          1982
          1982
          25 November 2008
          : 16
          : 1
          : 23-31
          Affiliations
          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
          Article
          179481 Horm Res 1982;16:23–31
          10.1159/000179481
          6279481
          80300cb2-ac89-41f1-a840-9d69d21c24b9
          © 1982 S. Karger AG, Basel

          Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

          History
          Page count
          Pages: 9
          Categories
          Paper

          Endocrinology & Diabetes,Neurology,Nutrition & Dietetics,Sexual medicine,Internal medicine,Pharmacology & Pharmaceutical medicine
          Hypercorticosteronism,Rokitansky-Kuster-Hauser syndrome,Impuberism,17ߝ20-Desmolase,Female pseudohermaphroditism,FoUicular cyst,17α-Hydroxylase

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