Trial record 5 of 11 for:    "Persistent Hyperinsulinemia Hypoglycemia of Infancy" OR "familial hyperinsulinism"

Effect of Exendin-(9-39) on Glycemic Control in Subjects With Congenital Hyperinsulinism

This study is ongoing, but not recruiting participants.
Sponsor:
Collaborators:
Information provided by (Responsible Party):
Diva De Leon, Children's Hospital of Philadelphia
ClinicalTrials.gov Identifier:
NCT00571324
First received: December 10, 2007
Last updated: July 30, 2014
Last verified: July 2014
  Purpose

The purpose of this study is to determine if Exendin-(9-39), an antagonist of the glucagon-like peptide-1 (GLP-1) receptor with effects on the pancreatic beta cell, increases fasting blood glucose levels in subjects with congenital hyperinsulinism.


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

Study Type: Interventional
Study Design: Intervention Model: Crossover Assignment
Masking: Open Label
Official Title: An Open Label Pilot Study of the Effects of the Glucagon-like Peptide-1 Receptor Antagonist, Exendin-(9-39) on Glycemic Control in Subjects With Congenital Hyperinsulinism

Resource links provided by NLM:


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

Primary Outcome Measures:
  • Fasting blood glucose level [ Time Frame: 7 hours ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • Insulin, glucagon-like peptide-1 and insulin levels [ Time Frame: 7 hours ] [ Designated as safety issue: No ]

Estimated Enrollment: 10
Study Start Date: August 2007
Estimated Study Completion Date: December 2014
Estimated Primary Completion Date: December 2014 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Experimental: 1 Drug: exendin-(9-39)
A short term intravenous infusion of exendin-(9-39) over 6 hours.

Detailed Description:

This is an open-label, pilot study , to determine if Exendin-(9-39), an antagonist of the glucagon-like peptide-1 (GLP-1) receptor with effects on the pancreatic beta cells, increases fasting blood glucose levels in subjects with congenital hyperinsulinism. Our overall hypothesis is that abnormal GLP-1 secretion resulting from dysfunctional nutrient sensing in intestinal L-cells plays a role in the dysregulated insulin secretion characteristic of this disorder, and that antagonism of the GLP-1 receptor will increase fasting blood glucose levels.

Aim 1. To evaluate the dose of exendin-(9-39) required to elevate fasting blood glucose levels in subjects with congenital hyperinsulinism due to KATP channel mutations.

Aim 2. To determine therapeutic plasma levels, plasma half-life and pharmacokinetics of exendin-(9-39) during an intravenous short-term infusion in subjects with congenital hyperinsulinism due to KATP channel mutations.

  Eligibility

Ages Eligible for Study:   7 Years to 60 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • Subjects with congenital hyperinsulinism

Exclusion Criteria:

  • Acute medical illness
  • History of other systemic chronic disease such as cardiac failure, renal insufficiency, hepatic insufficiency, chronic obstructive pulmonary disease, anemia, or uncontrolled hypertension
  • Pregnancy
  • Diabetes mellitus
  • Use of medications that affect glucose metabolism, such as glucocorticoids, beta agonists, diazoxide and octreotide.
  • Subjects will be eligible to participate 48 hrs after the last dose of octreotide and 72 hrs after last dose of diazoxide
  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: NCT00571324

Locations
United States, Pennsylvania
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, United States, 19104
Sponsors and Collaborators
Diva De Leon
Investigators
Principal Investigator: Diva D De Leon, MD Children's Hospital of Philadelphia
  More Information

No publications provided by Children's Hospital of Philadelphia

Additional publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
Responsible Party: Diva De Leon, M.D. Assistant Professor of Pediatrics, Children's Hospital of Philadelphia
ClinicalTrials.gov Identifier: NCT00571324     History of Changes
Other Study ID Numbers: 2007-1-5131, R03DK078535-01
Study First Received: December 10, 2007
Last Updated: July 30, 2014
Health Authority: United States: Food and Drug Administration

Keywords provided by Children's Hospital of Philadelphia:
hyperinsulinism
hypoglycemia
KATP channel
SUR-1
Kir6.2

Additional relevant MeSH terms:
Congenital Hyperinsulinism
Hyperinsulinism
Glucose Metabolism Disorders
Metabolic Diseases
Pancreatic Diseases
Digestive System Diseases
Infant, Newborn, Diseases
Hypoglycemia
Glucagon-Like Peptide 1
Incretins
Hormones
Hormones, Hormone Substitutes, and Hormone Antagonists
Physiological Effects of Drugs
Pharmacologic Actions

ClinicalTrials.gov processed this record on August 21, 2014