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      Treatment of Growth Hormone Deficiency in Very Young Children

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          Abstract

          Growth hormone (GH) deficiency is a rare disease in very young children and a challenge to the physician in terms of clinical recognition, diagnosis and treatment. Here, we review the available information regarding substitution of GH and other pituitary hormones in this patient group. Our results confirm the severity of the clinical presentation and the rapid loss of height (measured in standard deviation scores) in hypopituitary patients that occurs early in life. GH therapy induces a rapid catch-up of growth and the frequency of injections appears to be a more important determinant than the size of the dose itself. Long-term results are available in only a small percentage of patients, yet they show a favourable auxological outcome, compared with target height or historical height data from patients with severe hypopituitarism. In conclusion, although tremendous progress has been made in providing for those children with early onset and severe hypopituitarism, there is still a long way to go before we can claim that we have completely normalized their condition. In particular, careful assessments of the psychological and neurodevelopmental outcomes will become necessary in order to evaluate the ‘final results’ of endocrine replacement therapies.

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          Most cited references 8

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          Serum levels of insulin-like growth factor (IGF)-binding protein-3 (IGFBP-3) in healthy infants, children, and adolescents: the relation to IGF-I, IGF-II, IGFBP-1, IGFBP-2, age, sex, body mass index, and pubertal maturation

           A Juul (1995)
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            Reference Values for IGF-I throughout Childhood and Adolescence: A Model that Accounts Simultaneously for the Effect of Gender, Age, and Puberty

             C Löfqvist (2001)
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              Endocrinopathies associated with midline cerebral and cranial malformations.

              We systematically reviewed a series of patients (n = 85) with midline cerebral and cranial malformations to correlate the endocrinopathy with the neuroanatomic defect. Midline cleft lip and palate was associated not only with growth hormone deficiency (GHD) but also with diabetes insipidus (DI); holoprosencephaly and optic nerve hypoplasia with absence of the septum pellucidum had a similar incidence of GHD and DI. Optic nerve hypoplasia with absence of the septum pellucidum had the highest incidence of multiple pituitary endocrinopathies and of neonatal hypoglycaemia. Unilateral, although more commonly bilateral, optic nerve hypoplasia was associated with GHD.
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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
                978-3-8055-7609-3
                978-3-318-00998-9
                1663-2818
                1663-2826
                2003
                July 2003
                17 November 2004
                : 60
                : Suppl 1
                : 10-17
                Affiliations
                aDepartment of Paediatric Endocrinology and INSERM U561, Groupe hospitalier Cochin-Saint Vincent de Paul and Faculté Cochin, Université Paris V, Paris; bDepartment of Pediatrics, Dijon, France
                Article
                71220 Horm Res 2003;60(suppl 1):10–17
                10.1159/000071220
                12955012
                © 2003 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: 4, Tables: 1, References: 35, Pages: 8
                Categories
                GHD from Birth to 2 Years of Age

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