Effect of Exendin-(9-39) on Fasting Adaptation and Protein Sensitivity

This study is currently recruiting participants. (see Contacts and Locations)
Verified March 2016 by Children's Hospital of Philadelphia
Information provided by (Responsible Party):
Diva De Leon, Children's Hospital of Philadelphia
ClinicalTrials.gov Identifier:
First received: May 8, 2009
Last updated: March 7, 2016
Last verified: March 2016
The purpose of this study is to examine the effects of exendin-(9-39) on fasting blood glucose and protein induced hypoglycemia on subjects with Congenital Hyperinsulinism. Funding Source - FDA OODP.

Condition Intervention Phase
Congenital Hyperinsulinism
Drug: Exendin-(9-39)
Drug: placebo
Phase 1
Phase 2

Study Type: Interventional
Study Design: Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: Open Label
Primary Purpose: Treatment
Official Title: Role of GLP-1 in Congenital Hyperinsulinism:Effect of Exendin-(9-39) on Fasting Adaptation and Protein Sensitivity

Resource links provided by NLM:

Further study details as provided by Children's Hospital of Philadelphia:

Primary Outcome Measures:
  • Blood Glucose Levels [ Time Frame: -60, 0, and every 30 minutes for 6 hours ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • Plasma Insulin, C-Peptide, glucagon and intact GLP-1 levels [ Time Frame: -60, 0, and every 30 minutes for 6 hours ] [ Designated as safety issue: No ]

Estimated Enrollment: 18
Study Start Date: May 2009
Estimated Study Completion Date: April 2017
Estimated Primary Completion Date: April 2017 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Placebo Comparator: Placebo
at time 0,placebo(0.9%NaCl) at a dose ranging from 100-500pmol/kg/min will be started and continue for 6 hours.
Drug: placebo
placebo (0.9% NaCl)
Other Name: (0.9% NaCl)
Experimental: Exendin-(9-39)
at time 0,exendin-(9-39) at a dose ranging from 100-500pmol/kg/min will be started and continue for 6 hours.
Drug: Exendin-(9-39)

Detailed Description:
This is a placebo controlled study with randomized crossover design to evaluate the effect of the glucagon-like peptide-1 (GLP-1) receptor antagonist, exendin-(9-39), on fasting blood glucose levels, protein-induced hypoglycemia, and fasting tolerance of subjects with congenital hyperinsulinism due to mutations in the ATP- sensitive potassium channel (KATP) channel.

Ages Eligible for Study:   6 Months to 18 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No

Inclusion Criteria:

  • Confirmed diagnosis of hyperinsulinism
  • Mutation analysis results demonstrating KATP channel defect
  • Age 6 months to 18 years with
  • Persistent hypoglycemia

Exclusion Criteria:

  • Current therapy with medications that may affect glucose metabolism such as octreotide, diazoxide, high dose glucocorticoids, adrenergic agents, etc. Subjects will be eligible to participate if the last dose of octreotide is given 48 hrs before study day 1 and the last dose of diazoxide is given 72 hours before study day 1
  • Evidence of a medical condition that might alter results or compromised the elimination of the peptide, including active infection, kidney failure, severe liver dysfunction, severe respiratory or cardiac failure
  • Pregnancy
  • Subjects with milk protein allergy will be excluded for participating in studies involving protein tolerance test
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00897676

Contact: Stephanie Givler, BS, CCRC 267-426-7622 givler@email.chop.edu

United States, Pennsylvania
The Children's Hospital of Philadelphia Recruiting
Philadelphia, Pennsylvania, United States, 19104
Contact: Stephanie Givler, BS    267-426-7622    givler@email.chop.edu   
Principal Investigator: Diva D De Leon, MD         
Sub-Investigator: Charles A Stanley, MD         
Sponsors and Collaborators
Diva De Leon
Principal Investigator: Diva D DeLeon, MD Children's Hospital of Philadelphia
  More Information

Responsible Party: Diva De Leon, M.D. Assistant Professor of Pediatrics, Children's Hospital of Philadelphia
ClinicalTrials.gov Identifier: NCT00897676     History of Changes
Other Study ID Numbers: 2008-10-6255  R01FD004905 
Study First Received: May 8, 2009
Last Updated: March 7, 2016
Health Authority: United States: Food and Drug Administration
United States: Institutional Review Board

Keywords provided by Children's Hospital of Philadelphia:
KATP channel

Additional relevant MeSH terms:
Congenital Hyperinsulinism
Digestive System Diseases
Glucose Metabolism Disorders
Infant, Newborn, Diseases
Metabolic Diseases
Pancreatic Diseases

ClinicalTrials.gov processed this record on May 23, 2016