CHI Congenital hyperinsulinism
Conditions
Interventions
Sponsors
Nucleaire geneeskunde en radiologie
Eligibility
Age
2 Years to 17 Years
Inclusion criteria
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 PET/CT) not older than 8 weeks •
Exclusion criteria
Exclusion criteria: • Genetically proven diffuse CHI (presenting with a homozygous or compound heterozygous ABCC8/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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The expression and distribution of the GLP-1R in the pancreas of children ( | — |
Secondary
| Measure | Time frame |
|---|---|
| • Comparison of the sensitivity of 68Ga-NODAGA-exendin 4 PET/Ct and 18F-DOPA PET/CT for the pre-operative localization of focal CHI and the discrimination between focal and diffuse CHI. • Analysis of the kinetics of radiotracer uptake in the pancreas of CHI patients. • Determination of the minimal injected dose of 68Ga-NODAGA-exendin 4 needed for accurate imaging. • Determination of the effective radiation dose received by children injected with the calculated minimum dose of 68Ga-NODAGA-exendin 4. • Assessment of the safety (side effects) of 68Ga-NODAGA-exendin 4 as compared to 18F-DOPA. • Calculation and comparison of the interobserver variability of 68Ga-NODAGA-exendin 4 PET/CT and 18F-DOPA PET/CT • Evaluation of the clinical outcome parameters (laboratory parameters (glucose) and dosage of medical treatment) after surgery | — |
Countries
Netherlands
Outcome results
None listed