The physiology of glucagon-like peptide-l receptor expression in patients with endogenous hyperinsulinism: correlation with histopathology

Phase 2

Conditions: CHI
Congenital hyperinsulinism
10014699
10018424

Registration Number: NL-OMON46337

Lead Sponsor: ucleaire geneeskunde en radiologie

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Pending

Sex: Not specified

Target Recruitment: 5

Inclusion Criteria

- Biochemically and clinically proven endogenous congenital hyperinsulinism:
- Unresponsive to medical treatment (diazoxide)
- Indication for 18F-DOPA PET/CT based on mutation analysis
- Standard imaging (18F-DOPA PETICT) not older than 8 weeks
- <16 years old;- informed consent signed by parents or legal guardians of the patient.

Exclusion Criteria

- Genetically proven diffuse CHI (presenting with a homozygous or compound heterozygous ABCCS/KCNJ11 mutation)
- Calculated creatinine clearance below 40 ml/min
- Evidence of other malignancy than insulin producing tumors in conventional imaging (suspicious liver, bone and lung lesions based on CT)
- Age >16 years
- No signed informed consent

Study & Design

Study Type: Interventional

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>The expression and distribution of the GLP-1R in the pancreas of children (<16<br /><br>years) with proven endogenous congenital hyperinsulinism by comparison of<br /><br>68Ga-NODAGA-exendin 4 PET/CT imaging data with autoradiography and histology<br /><br>performed on specimens collected during surgery.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>- Comparison of the sensitivity of 68Ga-NODAGA-exendin 4 PET/CT and 18F-DOPA<br /><br>PET/CT for the pre-operative localization of focal CHI and the discrimination<br /><br>between focal and diffuse CHl.<br /><br>- Determination of the minimal injected dose of 68Ga-NODAGA-exendin 4 needed<br /><br>for accurate imaging.<br /><br>- Determination of the effective radiation dose received by children injected<br /><br>with the calculated minimum dose of 68Ga-NODAGA-exendin 4.<br /><br>- Assessment of the safety (side effects) of 68Ga-NODAGA-exendin 4 as compared<br /><br>to 18F-DOPA.<br /><br>- Calculation and comparison of the interobserver variability of<br /><br>68Ga-NODAGA-exendin 4 PET/CT and 18F-DOPA PET/CT<br /><br>- Evaluation of the clinical outcome parameters (laboratory parameters<br /><br>(glucose) and dosage of medical treatment) after surgery</p><br>