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      Metabolic Status of Children with Growth Hormone Insensitivity Syndrome and Responses to Treatment with IGF-I

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          Abstract

          Studies to date on the treatment with insulin-like growth factor I (IGF-I) of children with growth hormone (GH) insensitivity syndrome (GHIS) have concentrated principally on the effects of IGF-I therapy on the GH-IGF-I axis and on growth velocity. Little is known, however, about the metabolic status of children with GHIS and the consequences of IGF-I treatment on circulating intermediary metabolites involved in glucose homeostasis. We have studied 5 children with GHIS, aged 4.5–9 years, 3 male and 2 female, before and after 3 months of treatment with recombinant IGF-I, 80 µg/kg s.c. twice a day. The children were short (height SDS –3.8 to –7.3) and growing slowly (height velocity 1.8–3.4 cm/year). All were neurodevelopmentally normal at the time of the study with no history of severe symptomatic hypoglycaemia, in particular during the neonatal period. Before treatment, 4 of the 5 children demonstrated spontaneous hypoglycaemia (blood glucose <2.6 mmol/l), particularly at night, accompanied by substantial hyperketonaemia (range 1.43–4.63 mmol/l) and hyperfattyacidaemia (range 1.11–3.08 mmol/l). After 3 months of IGF-I treatment, the blood glucose concentrations in the diurnal profile were increased, with improvement in spontaneous hypoglycaemia and with increased fasting tolerance. The postprandial insulin-to-glucose ratio was reduced. GHIS is thus associated with asymptomatic nocturnal hypoglycaemia without apparent neurological deficit. Despite functional GH deficiency, brisk counter-regulation to hypoglycaemia occurred as evidenced by hyperfattyacidaemia and hyperketonaemia. Treatment with IGF-I twice a day improved fasting glucose tolerance, diurnal blood glucose concentrations, and postprandial insulin-to-glucose ratios. There was no exacerbation of hypoglycaemia on treatment. We suggest that in severe GH resistance, a protective mechanism exists for the brain from the effects of hypoglycaemia, at least partially mediated by excessive production of counter-regulatory metabolic fuels.

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          The insulin-like growth factor (IGF)-binding proteins and IGF bioactivity in Laron-type dwarfism

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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
            1998
            August 1998
            17 August 1998
            : 50
            : 2
            : 61-70
            Affiliations
            a London Centre for Paediatric Endocrinology and Metabolism at Great Ormond Street Hospital for Children and Institute of Child Health, London; b Paediatric Endocrinology Section, Department of Endocrinology, St. Bartholomew’s Hospital, London, UK
            Article
            23236 Horm Res 1998;50:61–70
            10.1159/000023236
            9701698
            © 1998 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
            Pages: 10
            Categories
            Original Paper

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