Journal Mobile Options
Table of Contents
Vol. 70, No. 1, 2008
Issue release date: July 2008
Section title: Novel Insights from Clinical Practice
Horm Res 2008;70:59–64
(DOI:10.1159/000129680)

Long-Term Non-Surgical Therapy of Severe Persistent Congenital Hyperinsulinism with Glucagon

Mohnike K. · Blankenstein O. · Pfuetzner A. · Pötzsch S. · Schober E. · Steiner S. · Hardy O.T. · Grimberg A. · van Waarde W.M.
aDepartment of Pediatrics, Otto von Guericke University of Magdeburg and bCharité University of Medicine, Berlin, Germany; cPharmaceutical Discovery Corp., Elmsford, New York, N.Y., USA; dDepartment Pediatrics, University of Vienna, Austria; eDivision of Pediatric Endocrinology, The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa., USA, and fDepartment of Pediatric Endocrinology, University Hospital of Groningen, The Netherlands

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 38.00
Account: USD 26.50

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

Subscribe

  • Automatic perpetual access to all articles of the subscribed year(s)
  • Unlimited re-access via Subscriber Login or MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more

Subcription rates


Select


Article / Publication Details

First-Page Preview
Abstract of Novel Insights from Clinical Practice

Received: 8/15/2006
Accepted: 10/17/2007
Published online: 5/21/2008

Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 1

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP

Abstract

Background: Congenital hyperinsulinism (CHI) is characterized by severe hypoglycemia caused by dysregulated insulin secretion. The long-term outcome is dependent on prevention of hypoglycemic episodes to avoid the high risk of permanent brain damage. Severe cases are usually resistant to diazoxide or nifedipine. In addition, somatostatin analogues are ineffective in a subgroup of patients to achieve stable euglycemia. In these infants the only remaining long-term option has been subtotal pancreatectomy with high risk of diabetes mellitus. Intravenous infusions of glucagon are used as immediate treatment to stabilize euglycemia in affected newborns. The rationale of this treatment comes from the observation of an increased glycogen content of the liver when glycogenolysis is inhibited by insulin. Objective: To review the efficacy and safety of long-term subcutaneous glucagon infusion as a potential therapeutic option for blood glucose stabilization in infants with severe CHI without the need of additional intravenous glucose or immediate surgical intervention. Method: Retrospective review of 9 children with CHI who received continuous subcutaneous infusion of glucagon for weeks or months. Glucagon was added to octreotide to replace octreotide-induced suppression of endogenous glucagon secretion, thereby liberating glucose by stimulation of hepatic glycogenolysis. In 3 cases, a stabilized formulation of glucagon was used to prevent glucagon crystallization that frequently occurs in smaller volumes. Results: Introduction of glucagon allowed the reduction or discontinuation of central glucose infusion in all children studied. In 2 patients, glucagon was introduced due to recurrent hypoglycemia despite subtotal pancreatectomy. Six out of 9 children were discharged home on this treatment, which their parents were able to continue without further symptomatic hypoglycemia, convulsions or unconsciousness. In 3 children, subcutaneous glucagon was continuously administered for 1–4 years leading to stable euglycemia. However, 2 children with diffuse type still required subtotal pancreatectomy. As a possible side effect, 2 children developed erythema necrolyticum, which resolved after discontinuation of the glucagon infusion. This has been described before in glucagonoma. Conclusion: In this retrospective series, combination therapy of low-dose octreotide and subcutaneous glucagon infusion has been effective in preventing hypoglycemic episodes in severe CHI. We propose this may serve as a therapeutic option in place of high rates of glucose infusion through a central venous catheter and as an alternative to subtotal pancreatectomy in diffuse type of CHI.


  

Author Contacts

Dr. Klaus L. Mohnike
Department of Pediatrics, Otto von Guericke University
Leipziger Strasse 44, DE–39120 Magdeburg (Germany)
Tel. +49 391 67 24031, Fax +49 391 67 290038
E-Mail susann.empting@med.ovgu.de

  

Article Information

Received: August 15, 2007
Accepted: October 17, 2007
Published online: May 21, 2008
Number of Print Pages : 6
Number of Figures : 0, Number of Tables : 1, Number of References : 40

  

Publication Details

Hormone Research (From Developmental Endocrinology to Clinical Research)

Vol. 70, No. 1, Year 2008 (Cover Date: July 2008)

Journal Editor: Czernichow P. (Paris)
ISSN: 0301–0163 (Print), eISSN: 1423–0046 (Online)

For additional information: http://www.karger.com/HRE


Article / Publication Details

First-Page Preview
Abstract of Novel Insights from Clinical Practice

Received: 8/15/2006
Accepted: 10/17/2007
Published online: 5/21/2008

Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 1

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP


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.