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      Cognitive Outcome in Adult Women Affected by Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

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          Abstract

          Background: Some research suggests that girls with congenital adrenal hyperplasia (CAH), who are exposed to higher than normal levels of prenatal androgens, perform better on spatial tasks, worse on verbal tasks and have a greater incidence of left-handedness than unaffected controls, all of which suggests the development of a more male-typical cognitive pattern. However, research in all three areas has produced inconsistent findings. Objectives: To determine if prenatal androgen exposure has an organizing effect on female cognitive development and to what extent. Methods: 24 women, 21–71 years, with either the salt-losing (SL) or simple virilizing (SV) forms of CAH due to 21-hydroxylase deficiency, and 18 controls, 21–73 years, who were unaffected female relatives or women with polycystic ovary syndrome, were assessed with IQ, handedness, executive function, verbal learning and memory, non-verbal learning and memory, perceptual speed, visuospatial processing and visuomotor ability measures. The battery included tests known to elicit sex differences and control measures. Results: The findings did not support the hypothesis that women with CAH develop a more male-typical cognitive pattern. Conclusion: This study differs from others in the older age of its participants, grouping by SL/SV diagnosis and assessment of medical treatment and compliance as determined through hormone assays. Our findings provide additional support for the conclusion that, in adult women with CAH, previous prenatal androgen exposure does not enhance spatial abilities, impair verbal abilities nor alter hand preferences in a long-lasting way.

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          Most cited references 28

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          Magnitude of sex differences in spatial abilities: a meta-analysis and consideration of critical variables.

          In recent years, the magnitude, consistency, and stability across time of cognitive sex differences have been questioned. The present study examined these issues in the context of spatial abilities. A meta-analysis of 286 effect sizes from a variety of spatial ability measures was conducted. Effect sizes were partitioned by the specific test used and by a number of variables related to the experimental procedure in order to achieve homogeneity. Results showed that sex differences are significant in several tests but that some intertest differences exist. Partial support was found for the notion that the magnitude of sex differences has decreased in recent years. Finally, it was found that the age of emergence of sex differences depends on the test used. Results are discussed with regard to their implications for the study of sex differences in spatial abilities.
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            Rey-Osterrieth complex figure: normative values in an Italian population sample.

            The Rey-Osterrieth complex figure test (ROCF) is a neuropsychological test extensively used in clinical practice to investigate visuospatial constructional functions, visuographic memory and some aspects of planning and executive function. The aim of the present study was to collect normative values in an Italian normal population sample (n=280) for the direct copying and delayed (10 min) reproduction of the ROCF. Multiple regression analysis revealed significant effects of age and education on performance of both copying tasks, whereas sex appeared to affect only performance on the delayed copying task. Inferential cut-offs have been determined and equivalent scores computed. The availability of equivalent scores for the ROCF will prove useful in clinical assessment since it allows the comparison of a subject's performance on the ROCF with that on other neuropsychological tests for which normative values collected with similar methods are already available for the Italian population.
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              A redrawn Vandenberg and Kuse mental rotations test: different versions and factors that affect performance.

              The available versions of the Vandenberg and Kuse (1978) Mental Rotations Test (MRT) have physically deteriorated because only copies of copies are available. We report results from a redrawn version of the MRT and for alternate versions of the test. Males perform better than females, and students drawn from the physical sciences perform better than students drawn from the social sciences and humanities, confirming other reports with the original version of the MRT. Subjects find it very hard to perform the MRT when stimuli require rotation along both the top/bottom axis and the left/right axis. The magnitude of effect sizes for sex (which account, on average, for some 20% of the variance) does not increase with increasing difficulty of the task. Minimal strategy effects were observed and females did not perform differently during the menstrual period as opposed to the days between the menstrual periods. Practice effects are dramatic, confirming other reports with the original MRT, and can also be shown to be powerful in a transfer for practice paradigm, where test and retest involve different versions of the MRT. Main effects of handedness on MRT performance were not found.
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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
                2006
                March 2006
                29 March 2006
                : 65
                : 3
                : 142-150
                Affiliations
                aDivision of Pediatric Endocrinology, Johns Hopkins University School of Medicine, Baltimore, Md., bCounseling Psychology, School of Education, Lehigh University, Bethlehem, Pa., cDepartment of Epidemiology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Md., dDepartment of Psychology, Loyola College, Baltimore, Md., eDepartment of Biology, Drake University, Des Moines, Iowa, USA
                Article
                91793 Horm Res 2006;65:142–150
                10.1159/000091793
                16508325
                © 2006 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: 4, References: 47, Pages: 9
                Categories
                Original Paper

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