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      Growth and Metabolic Control during Puberty in Girls with X-Linked Hypophosphataemic Rickets

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          Abstract

          Objective: X-linked hypophosphataemic rickets (XLH) results in defective bone mineralization and impaired growth. Treatment with oral phosphate (Pi) and calcitriol improves but does not normalize growth. This study assessed whether pubertal growth and metabolic control contribute to the height deficit. Methods: Study included patients with XLH who were treated with Pi-calcitriol from diagnosis to adult height; their hospital records, biochemistry and radiographs were reviewed. Results: Six females with XLH were included. Their mean peak height velocity and total height gain during puberty were nearly normal despite deteriorating metabolic control. Conclusions: In treated girls with XLH, the pubertal growth is nearly normal despite suboptimal metabolic control. The major height loss occurs prior to puberty and is not recovered during the pubertal growth spurt.

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          Effect of growth hormone treatment on final height, phosphate metabolism, and bone mineral density in children with X-linked hypophosphatemic rickets.

          To evaluate the effect of growth hormone treatment on final height, phosphate metabolism, bone markers, and bone mineral density in children with X-linked hypophosphatemic rickets. Six patients (aged 7.8 +/- 1.8 years; height z score, -3.4 +/- 0.5) received conventional treatment (1,25-dihydroxyvitamin D(3) plus phosphate salts) combined with growth hormone (0.6-0.9 IU/kg per week, subcutaneously) (group A); 6 patients (aged 7.9 +/- 2.5 years; height z score, -2.1 +/- 0.6, P <.01 with respect to group A) received only conventional treatment (group B). Final height z score significantly improved in group A (-2.4 +/- 0.5, P <.03 with respect to the value at entry), whereas it did not change in group B (-2.8 +/- 0.5, P = NS). At final height, degree of body disproportion was similar between the groups (group A, 1.3 +/- 0.2; group B, 1.2 +/- 0.1; P = NS). Phosphate retention, bone markers, and radial bone mineral density increased only in group A. No adverse effects were observed. In poorly growing patients with X-linked hypophosphatemic rickets, growth hormone therapy combined with conventional treatment improves final height, phosphate retention, and radial bone mineral density, but it does not influence degree of body disproportion.
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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
            2004
            April 2004
            23 April 2004
            : 61
            : 5
            : 252-256
            Affiliations
            aDivision of Endocrinology and bDiagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, Ont., Canada; cDivision of Endocrinology, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
            Article
            77401 Horm Res 2004;61:252–256
            10.1159/000077401
            15031616
            © 2004 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: 21, Pages: 5
            Categories
            Original Paper

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