Hormone Research in Paediatrics
Juriaans A.F. · Trueba-Timmermans D.J. · Kerkhof G.F. ·
Grootjen L.N. · Walet S. · Sas T.C.J. · Rotteveel J. · Zwaveling-Soonawala N. ·
Verrijn Stuart A.A. · Hokken-Koelega A.C.S.
Log in to MyKarger to check if you already have access to this content.
Buy
FullText & PDF
Unlimited re-access via MyKarger
Unrestricted printing, no saving restrictions for personal use
read more
CHF 38.00 *
EUR 35.00 *
USD 39.00 *
Select
KAB
Buy a Karger Article Bundle (KAB) and profit from a discount!
If you would like to redeem your KAB credit, please log in.
Save over 20% compared to the individual article price.
Learn more
Rent via DeepDyve
Unlimited fulltext viewing of this article
Organize, annotate and mark up articles
Printing and downloading restrictions apply
Start free trial
Subscribe
Access to all articles of the subscribed year(s) guaranteed for 5 years
Unlimited re-access via Subscriber Login or MyKarger
Unrestricted printing, no saving restrictions for personal use
read more
Subcription rates
Select
* The final prices may differ from the prices shown due to specifics of VAT rules.
Article / Publication Details
Abstract
Introduction: Temple syndrome (TS14) is a rare imprinting disorder caused by maternal uniparental disomy of chromosome 14 (UPD(14)mat), paternal deletion of 14q32.2 or an isolated methylation defect. Most patients with TS14 develop precocious puberty. Some patients with TS14 are treated with growth hormone (GH). However, evidence for the effectiveness of GH-treatment in patients with TS14 is limited.
Methods: This study describes the effect of GH-treatment in 13 children and provides a subgroup analysis of 5 prepubertal children with TS14. We studied height, weight, body composition by Dual-Energy X-ray Absorptiometry (DXA), resting energy expenditure (REE) and laboratory parameters during 5 years of GH-treatment.
Results: In the entire group mean (95% CI) height SDS increased significantly during 5 years of GH-treatment from -1.78 (-2.52; -1.04) to 0.11 (-0.66; 0.87). Fat mass percentage (FM%) SDS decreased significantly during the first year of GH, and lean body mass (LBM) SDS and LBM index increased significantly during 5 years of treatment. IGF-1 and IGF-BP3 levels rose rapidly during GH-treatment and the IGF-1/IGF-BP3 molar ratio remained relatively low. Thyroid hormone levels, fasting serum glucose and insulin levels remained normal. In the prepubertal group, median (IQR) height SDS, LBM SDS and LBM index also increased. REE was normal at start and did not change during one year of treatment. Five patients reached adult height and their median (IQR) height SDS was 0.67 (-1.83; -0.01).
Conclusion: GH-treatment in patients with TS14 normalizes height SDS and improves body composition. There were no adverse effects or safety concerns during GH-treatment.
S. Karger AG, Basel
Article / Publication Details
Copyright / Drug Dosage / Disclaimer
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.
留言 (0)