Overview
Hyperinsulinemic hypoglycemia (also called congenital hyperinsulinism, persistent hyperinsulinemic hypoglycemia of infancy, or CHI) is a condition where the pancreas produces too much insulin, causing blood sugar levels to drop dangerously low. Insulin is the hormone that lowers blood sugar, so when too much is made — even when blood sugar is already low — the body cannot keep glucose at a safe level. The brain and other organs depend heavily on glucose for energy, so repeated or prolonged low blood sugar can cause serious harm, especially in babies and young children. The most common symptoms include shakiness, sweating, irritability, poor feeding, seizures, and in severe cases, loss of consciousness. In newborns, the signs can be subtle and easy to miss, which makes early diagnosis very important. Some forms are caused by changes (mutations) in specific genes that control how insulin is released from the pancreas, while other forms are linked to conditions like maternal diabetes or birth stress. Treatment ranges from frequent feeding and glucose supplementation to medications like diazoxide or octreotide, and in some cases surgery to remove part of the pancreas. With early diagnosis and proper management, many children do well, but without treatment, repeated low blood sugar episodes can lead to brain injury and developmental delays.
Key symptoms:
Low blood sugar (hypoglycemia)Shakiness or tremblingSweating, especially in infantsIrritability or unusual fussinessPoor feeding or refusing to eatSeizures or convulsionsPale or bluish skin colorLimpness or low muscle toneRapid heartbeatExtreme tiredness or lethargyConfusion or difficulty concentrating in older childrenLoss of consciousness in severe casesDevelopmental delays if untreated over time
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
5 eventsHanmi Pharmaceutical Company Limited — PHASE2
Zealand Pharma — PHASE3
Miguel Pampaloni — PHASE2
Cook Children's Health Care System — PHASE1
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Hyperinsulinemic hypoglycaemia.
5 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Hyperinsulinemic hypoglycaemia.
Community
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the specific cause of my child's hyperinsulinism — is it genetic, and which gene is involved?,Is this a focal or diffuse form, and how does that affect treatment options?,Should we do genetic testing on the whole family, and what does the result mean for future pregnancies?,What are the signs that diazoxide is working, and what should we do if it stops working?,What is our emergency plan if my child has a severe low blood sugar episode at home or at school?,What long-term monitoring does my child need for brain development, learning, and possible diabetes?,Are there clinical trials or specialized centers we should consider for our child's care?
Common questions about Hyperinsulinemic hypoglycaemia
What is Hyperinsulinemic hypoglycaemia?
Hyperinsulinemic hypoglycemia (also called congenital hyperinsulinism, persistent hyperinsulinemic hypoglycemia of infancy, or CHI) is a condition where the pancreas produces too much insulin, causing blood sugar levels to drop dangerously low. Insulin is the hormone that lowers blood sugar, so when too much is made — even when blood sugar is already low — the body cannot keep glucose at a safe level. The brain and other organs depend heavily on glucose for energy, so repeated or prolonged low blood sugar can cause serious harm, especially in babies and young children. The most common symptom
Are there clinical trials for Hyperinsulinemic hypoglycaemia?
Yes — 5 recruiting clinical trials are currently listed for Hyperinsulinemic hypoglycaemia on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Hyperinsulinemic hypoglycaemia?
25 specialists and care centers treating Hyperinsulinemic hypoglycaemia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.