Condition (MeSH)
Congenital Hyperinsulinism
ClinicalTrials.gov lists 38 trials for Congenital Hyperinsulinism or a narrower condition beneath it. Zealand Pharma has the most among industry and government lead sponsors, with 3.
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Trials per phase
1 more section with rows
37 of the 38 trials are tagged with Congenital Hyperinsulinism itself; the rest are tagged with a narrower condition beneath it.
When did Congenital Hyperinsulinism trials start? (17)
Shown in this order: most recent first, 1990 onwards. 2026 is partial: trials registered with a 2026 start date so far, not a full year.
Trials over time, by phase
- Phase 1
- Phase 1/2
- Phase 2
- Phase 2/3
- Phase 3
- Phase 4
- Not applicable
- Observational
Top of scale: 5
Trials registered with a start date in each year, 1990 onward, stacked by phase (complete years only; the current partial year is in the table's notes). The table below lists the same counts, most recent years first.
| Year | Trials started |
|---|---|
| 2026 (partial year) | 1 |
| 2025 | 1 |
| 2024 | 1 |
| 2022 | 2 |
| 2021 | 2 |
| 2020 | 2 |
| 2019 | 5 |
| 2018 | 2 |
| 2017 | 3 |
| 2016 | 4 |
Showing 10 of 17 earlier years.
No forecast is made for this condition: it averages 1.0 trial starts a year over the five years to September, under the 5 a year the method needs.
Recruitment duration, a model prediction: 2 trials still running at the 2026-10-09 snapshot, fewer than the 5 needed for a median. Source: ClinicalTrials.gov / AACT (CTTI), public domain.
What phase are Congenital Hyperinsulinism trials in? (9)
Shown in this order: phase order.
Show the trials by phase as a table
| Phase | Trials |
|---|---|
| Phase 1 | 5 |
| Phase 1/2 | 3 |
| Phase 2 | 12 |
| Phase 2/3 | 1 |
| Phase 3 | 5 |
| Phase 4 | 1 |
| Not applicable | 5 |
| Observational | 3 |
| Expanded access | 3 |
How many Congenital Hyperinsulinism trials are recruiting? (9)
Shown in this order: the order a trial moves through, from not yet recruiting to unknown.
| Status | Trials |
|---|---|
| Not yet recruiting | 1 |
| Recruiting | 3 |
| Active, not recruiting | 3 |
| Available | 1 |
| Completed | 22 |
Showing 5 of 9 statuses.
Status as registered on ClinicalTrials.gov. Each trial is counted once.
Which lead sponsors run the most Congenital Hyperinsulinism trials? (3)
Shown in this order: most trials first. Lead sponsors the registry classes as industry, NIH, US federal, other government or network. A hospital or university the registry files as government can appear here. Sponsors it classes as academic or other are shown on the full page, because that class cannot separate an institution from a named investigator.
| Sponsor | Trials |
|---|---|
| Zealand Pharma | 3 |
| Xeris Pharmaceuticals | 2 |
| Hanmi Pharmaceutical Company Limited | 1 |
Which drugs are being tested for Congenital Hyperinsulinism? (3)
Shown in this order: most trials first. One row per drug, matched to a single molecule under all its names, ranked by number of trials.
| Drug | Trials |
|---|---|
| 18F-DOPA | 4 |
| Avexitide | 4 |
| Dasiglucagon | 4 |
Interventions as named in the registry (12)
Shown in this order: most trials first.
| Drug | Trials |
|---|---|
| 18 F-DOPA | 2 |
| Pasireotide s.c. | 2 |
| Furosemide-IR | 1 |
| RZ358 Sequential Group Cohort 2 | 1 |
| r-glucagon | 1 |
Showing 5 of 12 interventions.
These are the names sponsors entered on ClinicalTrials.gov that we have not matched to a single molecule. Some are drugs under a brand or code name; others are procedures, doses or arm labels.
Which serious adverse events do Congenital Hyperinsulinism trials report? (10)
Shown in this order: most subjects affected first. Subjects affected summed across trials, a cross-trial signal, not a rate.
| Adverse event | Subjects affected | Trials |
|---|---|---|
| Acute respiratory failure | 2 | 1 |
| Respiratory distress | 2 | 1 |
| Eosinophilic Leukocytosis | 1 | 1 |
| Folliculitis | 1 | 1 |
| H1N1 Influenza | 1 | 1 |
Showing 5 of 10 adverse events.
Which publications cover Congenital Hyperinsulinism? (24)
Shown in this order: major-topic papers first, then newest, then most linked to trials.
| Publication | Journal | Year | Major topic |
|---|---|---|---|
| Global, multi-center, repeat-dose, phase 2 study of RZ358 (ersodetug), an insulin receptor antibody, for congenital hyperinsulinism. | Med (New York, N.Y.) | 2025 | Yes |
| Congenital hyperinsulinism: localization of a focal lesion with <sup>18</sup>F-FDOPA positron emission tomography. | Pediatric radiology | 2022 | Yes |
| Exendin-(9-39) Effects on Glucose and Insulin in Children With Congenital Hyperinsulinism During Fasting and During a Meal and a Protein Challenge. | Diabetes care | 2022 | Yes |
| Localized islet nuclear enlargement hyperinsulinism (LINE-HI) due to ABCC8 and GCK mosaic mutations. | European journal of endocrinology | 2022 | Yes |
| <sup>18</sup>F-6-Fluoro-l-Dopa PET/CT Imaging of Congenital Hyperinsulinism. | Journal of nuclear medicine : official publication, Society of Nuclear Medicine | 2021 | Yes |
Showing 5 of 24 publications.
Prevalence (16)
Shown in this order: jurisdiction, then disease name. Orphanet observation for the mapped ORPHA concept; the mapping relation column says whether that concept is exactly this condition or broader or narrower.
| Orphanet disease | Type | Class | Geography | Jurisdiction | Mapping relation |
|---|---|---|---|---|---|
| Congenital isolated hyperinsulinism | Prevalence at birth | 1-9 / 100 000 | United Kingdom | GB | Exact |
| Congenital isolated hyperinsulinism | Prevalence at birth | 1-9 / 100 000 | United Kingdom | GB | Exact |
| Congenital isolated hyperinsulinism | Prevalence at birth | 1-9 / 100 000 | Worldwide | Not stated | Exact |
| Congenital isolated hyperinsulinism | Prevalence at birth | 1-9 / 100 000 | Japan | Not stated | Exact |
| Congenital isolated hyperinsulinism | Prevalence at birth | 1-9 / 100 000 | Netherlands | Not stated | Exact |
Showing 5 of 16 prevalence observations.
Rare-disease identity (2)
Shown in this order: exact, broader, narrower, other.
| Orphanet disease | ORPHA code | Relation | Source |
|---|---|---|---|
| Congenital isolated hyperinsulinism | 657 | Exact | Orphanet Rare Disease Ontology (ORDO) |
| Congenital isolated hyperinsulinism | 657 | Exact | Orphadata Science knowledge files, Product 1 |
Sub-conditions in MeSH (1)
MeSH descendants one level down with at least one trial, most trials first.
Not on this page
Not measured: delivery record.