Management of Males with 45,X/46,XY Gonadal Dysgenesis

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Abstract

Males with the 45,X/46,XY karyotype and malformations of the external genitalia carry an increased risk of developing germ cell neoplasia of the gonads. We have studied gonadal tissue from 10 individuals, 0.3–17 years of age, with a male phenotype and either hypospadias and/or cryptorchidism. Four patients, 0.3–15 years of age, had carcinoma in situ, 1 boy had Sertoli-cell-only pattern and the remainder prepubertal histology. Gonadoblastoma or invasive carcinoma was not found. On the basis of our current knowledge we propose a strategy for management and follow-up of these boys in order to detect possible premalignant histological changes early and prevent development of a gonadal tumour.

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Journal

Journal ID (publisher-id): HRE

Journal ID (nlm-ta): Horm Res Paediatr

Journal ID (doi): 10.1159/issn.1663-2818

Title: Hormone Research in Paediatrics

Publisher: S. Karger AG

ISSN (Print): 1663-2818

ISSN (Electronic): 1663-2826

Publication date Subscription-year: 1999

Publication date (Print): July 1999

Publication date (Electronic): 26 January 2000

Volume: 52

Issue: 1

Pages: 11-14

Affiliations

^aDepartment of Growth and Reproduction GR, Rigshospitalet, Copenhagen, Denmark; ^bPaediatric Endocrinology Unit, Karolinska Hospital, Stockholm, Sweden; ^cDepartment of Paediatrics, Malmö University Hospital, University of Lund, Sweden, and ^dDepartment of Paediatrics, Østra Hospital, University of Gothenburg, Sweden

Article

Publisher ID: 23425 Other ID: Horm Res 1999;52:11–14

DOI: 10.1159/000023425

PubMed ID: 10640893

SO-VID: 2ee69376-1296-4663-b601-b0202b58c60e

License:

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

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Figures: 3, Tables: 1, References: 33, Pages: 4

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