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      Congenital Adrenal Hyperplasia: A Lifelong Disorder

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          Background: Congenital adrenal hyperplasia (CAH), the most common cause of ambiguous genitalia of the newborn, requires rapid assessment by a multidisciplinary team including a neonatologist, paediatric endocrinologist, paediatric urologist and geneticist. There is also a role for the clinical psychologist with psychosexual counselling experience as families cope with disorders of sex development. This brief review summarises the continuum of disorders that are manifested in patients with CAH according to age and sex, with emphasis on the lifetime nature of the issues that accompany this disorder and on the long-lasting ramifications of pediatric management decisions for both males and females. Conclusions: There are many management aspects of caring for patients with CAH that clearly fall into the purview of paediatricians or adult-care physicians. There are also areas where responsibilities overlap and require several professionals providing coordinated care.

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          Most cited references 21

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          Consensus statement on management of intersex disorders.

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            Cognitive theories of early gender development.

            The contribution of cognitive perspectives (cognitive-developmental theory and gender schema theory) to a contemporary understanding of gender development is evaluated. Recent critiques of cognitive approaches are discussed and empirical evidence is presented to counter these critiques. Because of the centrality of early gender development to the cognitive perspective, the latest research is reviewed on how infants and toddlers discriminate the sexes and learn the attributes correlated with sex. The essence of cognitive approaches--emphasis on motivational consequences of gender concepts; the active, self-initiated view of development; and focus on developmental patterns-is highlighted and contrasted with social-cognitive views. The value of cognitive theories to the field is illustrated, and recommendations are made concerning how to construct comprehensive, integrative perspectives of gender development.
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              Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology.


                Author and article information

                Horm Res Paediatr
                Hormone Research in Paediatrics
                S. Karger AG
                December 2007
                10 December 2007
                : 68
                : Suppl 5
                : 84-89
                Department of Paediatrics, Addenbrooke’s Hospital, University of Cambridge, Cambridge, UK
                110585 Horm Res 2007;68:84–89
                © 2007 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.

                Page count
                Figures: 1, Tables: 2, References: 31, Pages: 6
                Pediatric Workshop 1


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