CHI Congenital hyperinsulinism
Conditions
Interventions
GLP-1 receptor PET/CT
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 PETICT) not older than 8 weeks -
Exclusion criteria
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
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 CHl. - 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