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      Introduction and Management of Puberty in Girls

      review-article
      Hormone Research in Paediatrics
      S. Karger AG
      Progestin, Therapy, Puberty, Girls, Estrogens

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          Abstract

          Background: Management of puberty in the hypogonadal girl remains controversial. Estrogens have profound effects on growth and development. Oral estrogens, the most widely used form, undergo first-pass metabolism in the liver and alter many aspects of hepatic function. Transdermal estrogens are not subject to first-pass metabolism and are effective at mimicking spontaneous pubertal estrogen levels resulting in normal pubertal development. Age-appropriate induction of puberty should be initiated at 12 to 13 years of age; delaying puberty any longer may compromise quality of life during adolescence. Estrogens should be started at a low dose, approximately one-tenth to one-eighth of the adult replacement dose, and then increased gradually over a period of 2 to 4 years. Generally, progestins are added after 2 years of estrogen therapy. With these treatment regimens, breast development typically proceeds at a normal pace; however, the effects on uterine dimensions are less clear. Concomitant treatment with oral dehydroepiandrosterone (or dehydroepiandrosterone sulfate) advances pubic hair development in the adolescent girl with panhypopituitarism. Conclusions: In the hypogonadal girl, puberty should be induced with very low doses of transdermal estradiol at 12 to 13 years of age with gradual dose escalations over 2 to 4 years. Combination estrogen/androgen therapy in girls with hypogonadism should be explored in future research.

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          Most cited references22

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          The effect of six months treatment with a 100 mg daily dose of dehydroepiandrosterone (DHEA) on circulating sex steroids, body composition and muscle strength in age-advanced men and women.

          The biological role of the adrenal sex steroid precursors--DHEA and DHEA sulphate (DS) and their decline with ageing remains undefined. We observed previously that administration of a 50 daily dose of DHEA for 3 months to age-advanced men and women resulted in an elevation (10%) of serum levels of insulin-like growth factor-I (IGF-I) accompanied by improvement of self-reported physical and psychological well-being. These findings led us to assess the effect of a larger dose (100 mg) of DHEA for a longer duration (6 months) on circulating sex steroids, body composition (DEXA) and muscle strength (MedX).
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            Effects of testosterone replacement in androgen-deficient women with hypopituitarism: a randomized, double-blind, placebo-controlled study.

            Hypopituitarism in women is characterized by profound androgen deficiency due to a loss of adrenal and/or ovarian function. The effects of testosterone replacement in this population have not been reported. The objective of the study was to determine whether physiologic testosterone replacement improves bone density, body composition, and/or neurobehavioral function in women with severe androgen deficiency secondary to hypopituitarism. This was a 12-month randomized, placebo-controlled study. The study was conducted at a general clinical research center. Fifty-one women of reproductive age with androgen deficiency due to hypopituitarism participated. Physiologic testosterone administration using a patch that delivers 300 microg daily or placebo was administered. Bone density, fat-free mass, and fat mass were measured by dual x-ray absorptiometry. Thigh muscle and abdominal cross-sectional area were measured by computed tomography scan. Mood, sexual function, quality of life, and cognitive function were assessed using self-administered questionnaires. Mean free testosterone increased into the normal range during testosterone administration. Mean hip (P = 0.023) and radius (P = 0.007), but not posteroanterior spine, bone mineral density increased in the group receiving testosterone, compared with placebo, as did mean fat-free mass (P = 0.040) and thigh muscle area (P = 0.038), but there was no change in fat mass. Mood (P = 0.029) and sexual function (P = 0.044) improved, as did some aspects of quality of life, but not cognitive function. Testosterone at physiologic replacement levels was well tolerated, with few side effects. This is the first randomized, double-blind, placebo-controlled study to show a positive effect of testosterone on bone density, body composition, and neurobehavioral function in women with severe androgen deficiency due to hypopituitarism.
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              Recommendations for the Diagnosis and Management of Turner Syndrome

              P Saenger (2001)
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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
                978-3-8055-8475-3
                978-3-8055-8476-0
                1663-2818
                1663-2826
                2007
                December 2007
                10 December 2007
                : 68
                : Suppl 5
                : 80-83
                Affiliations
                Erasmus Medical Centre, Sophia Children’s Hospital, Endocrinology Subdivision, Rotterdam, The Netherlands
                Article
                110584 Horm Res 2007;68:80–83
                10.1159/000110584
                18174716
                bd44f2c2-1764-4d05-ac0c-c5716e45ab50
                © 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.

                History
                Page count
                Tables: 1, References: 36, Pages: 4
                Categories
                Pediatric Plenary Session

                Endocrinology & Diabetes,Neurology,Nutrition & Dietetics,Sexual medicine,Internal medicine,Pharmacology & Pharmaceutical medicine
                Progestin,Therapy,Estrogens,Puberty,Girls

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