Cover

Nutrition and Growth

Editor(s): Shamir R. (Tel Aviv) 
Turck D. (Lille) 
Phillip M. (Tel Aviv) 
Table of Contents
Vol. 106, 2013
Section title: Paper
Shamir R, Turck D, Phillip M (eds): Nutrition and Growth. World Rev Nutr Diet. Basel, Karger, 2013, vol 106, pp 105–112
(DOI:10.1159/000342556)

Treatment Options for Children with Monogenic Forms of Obesity

Hainerová I.A. · Lebl J.
aDepartment of Pediatrics and Center for Research of Diabetes, Metabolism and Nutrition, Third Faculty of Medicine and bDepartment of Pediatrics, Second Faculty of Medicine, Charles University, Prague, Czech Republic

Do you have an account?

Register and profit from personalized services (MyKarger) Login Information

Please create your User ID & Password





Contact Information









I have read the Karger Terms and Conditions and agree.

Register and profit from personalized services (MyKarger) Login Information

Please create your User ID & Password





Contact Information









I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in

Buy

  • FullText & PDF
  • Unlimited re-access via MyKarger (new!)
  • Unrestricted printing, no saving restrictions for personal use
  • Reduced rates with a PPV account
read more

Direct: USD 33.00
Account: USD 23.00

Select

Rent/Cloud

  • Rent for 48h to view
  • Buy Cloud Access for unlimited viewing via different devices
  • Synchronizing in the ReadCube Cloud
  • Printing and saving restrictions apply

Rental: USD 8.50
Cloud: USD 20.00

Select

Complete book

  • Immediate access to all parts of this book
  • Cover-to-cover formats may be available
  • Unlimited re-access via MyKarger
  • Unrestricted printing, no saving restriction for personal use
read more

Pricing depends on hard-cover price


Select


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 2/11/2013
Cover Date: 2013

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 1

ISBN: 978-3-318-02265-0 (Print)
eISBN: 978-3-318-02266-7 (Online)

Abstract

Mutations in genes involved in energy balance regulation within the central nervous system lead to monogenic forms of obesities. Individuals with these mutations are characterized by early-onset obesity and in some cases by endocrine abnormalities. Carriers of leptin gene mutations are able to normalize their body weight after daily subcutaneous leptin administration. Pharmacotherapy targeting the specific-gene deficiencies has not clinically been tested in other monogenic obesities. Mutations in the melanocortin 4 receptor gene (MC4R) represent the most common monogenic cause of human obesity. Several treatment options have been investigated in subjects with MC4R mutations. Few studies showed that an intensive life-style intervention induces similar weight reduction in MC4R mutation carriers in comparison to MC4R mutation noncarriers. However, long-term body weight maintenance is hardly ever achieved in MC4R mutation carriers. Sibutramine, serotonin and noradrenalin reuptake inhibitor, in MC4R mutation carriers induced weight reduction and improved cardiometabolic health risks. This result was also found in our homozygous MC4R mutation carrier. In vitro studies of melanocortin agonists efficiently activate mutated MC4R with impaired endogenous agonist functional response and thus, further research in the development of drugs for MC4R mutations is needed. An administration of intranasal adrenocorticotropic hormone was not shown to be effective in subjects with pro-opiomelanocortin gene mutations. Bariatric surgery has also been performed in few of MC4R mutation carriers. After gastric banding, lower body weight reduction and worse improvement of metabolic complications was found in MC4R mutation carriers versus noncarriers. However, preliminary results suggest that diversionary operations as gastric bypass represent a suitable method also for MC4R mutation carriers. In conclusion, the management of monogenic obesities still remains a challenge.


  

Author Contacts

Irena Aldhoon Hainerová, MD, PhD
Department of Pediatrics and Center for Research of Diabetes, Metabolism and Nutrition
Third Faculty of Medicine, Charles University
Šrobárova 50, 100 34 Prague 10 (Czech Republic)
E-Mail ihainer@hotmail.com

  

Article Information

Published online: February 11, 2013
Number of Print Pages : 8

  

Publication Details

Book Serie: World Review of Nutrition and Dietetics, Vol. 106, Year 2013

Editor(s): Shamir, R. (Tel Aviv); Turck, D. (Lille); Phillip, M. (Tel Aviv)
ISSN: 0084-2230 (Print), eISSN: 1662-3975 (Online)

For additional information:
http://content.karger.com/ProdukteDB/produkte.asp?issn=0084-2230

Book Title: Nutrition and Growth

Editor(s): Shamir R, Turck D, Phillip M (eds)

For additional information:
http://content.karger.com/ProdukteDB/produkte.asp?issn=0084-2230&volume=106


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 2/11/2013
Cover Date: 2013

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 1

ISBN: 978-3-318-02265-0 (Print)
eISBN: 978-3-318-02266-7 (Online)


Copyright / Drug Dosage

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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.