Hyperinsulinism

Hyperinsulinism means that the body is making too much insulin compared with the amount of sugar (glucose) in the blood. Insulin is a hormone made by the beta cells in the pancreas. Its main job is to move sugar from the blood into the body’s cells, where it is used for energy or stored for later. In hyperinsulinism, insulin is released in an abnormal, uncontrolled, or excessive way. As a result, blood sugar can drop too low (hypoglycemia), especially in newborns, infants, and children, but it can also happen in adults. MDPI+1

Hyperinsulinism means the body makes too much insulin. Insulin is a hormone from the pancreas that normally keeps blood sugar in a safe range. In hyperinsulinism, insulin stays high even when blood sugar is low. This can cause repeated or severe low blood sugar (hypoglycemia), especially in babies and children. If low sugar is not treated, it can harm the brain. Treatment plans usually combine food strategies, medicines, sometimes surgery, and very close monitoring by a specialist team. Frontiers+1

When insulin is too high, the body uses up blood sugar too quickly and stops making new sugar in the liver. This combination can cause frequent or severe hypoglycemia. If low blood sugar is not recognized and treated, it can affect the brain, because the brain needs a constant supply of glucose to work properly. In babies and children, repeated low sugar episodes can lead to seizures and long-term brain problems. This is why hyperinsulinism is considered a serious condition that needs careful and fast medical attention. PMC+1

Hyperinsulinism can be congenital (present from birth because of genetic changes) or acquired (develops later because of tumors, medicines, or insulin resistance). In children, congenital hyperinsulinism is the most common cause of long-lasting hypoglycemia. In adults, insulin resistance and insulin-producing tumors are common reasons for high insulin levels. MDPI+1

Other Names for Hyperinsulinism

Hyperinsulinism is sometimes described or labeled with other names. Some terms are very close in meaning, and some are used mainly in babies:

• Hyperinsulinemia – general term for higher-than-normal insulin levels in the blood, often used in adults with insulin resistance. Cleveland Clinic+1

• Hyperinsulinemic hypoglycemia – low blood sugar caused by too much insulin, used for both children and adults. Wikipedia

• Congenital hyperinsulinism (CHI) – hyperinsulinism present from birth due to genetic or developmental problems in the beta cells. rarediseases.org+1

• Persistent hyperinsulinemic hypoglycemia of infancy – older name for congenital hyperinsulinism in babies. e-apem.org+1

• Nesidioblastosis – older term for diffuse overgrowth and overactivity of beta cells in the pancreas; now usually included under congenital hyperinsulinism. Physiology Journals+1

Types of Hyperinsulinism

Doctors group hyperinsulinism into types based on when it appears, how the pancreas looks, and the cause. Common types include:

1. Congenital diffuse hyperinsulinism – all or most of the beta cells in the pancreas release too much insulin; usually due to genetic changes. MDPI+1

2. Congenital focal hyperinsulinism – only a small area (focus) in the pancreas has abnormal beta cells that overproduce insulin; that part can sometimes be removed by surgery. PMC+1

3. Transient neonatal hyperinsulinism – occurs in newborns for a short time (days to weeks), often due to stress around birth such as low oxygen or poor growth in the womb; usually improves with time. NCBI+1

4. Syndromic hyperinsulinism – hyperinsulinism that is part of a larger genetic syndrome, such as Beckwith–Wiedemann syndrome or some overgrowth and developmental syndromes. Frontiers+1

5. Insulinoma-related hyperinsulinism – caused by a small tumor of beta cells (insulinoma) that releases insulin in large amounts, more common in adults. ScienceDirect+1

6. Post–bariatric surgery hyperinsulinism – after weight-loss surgery, some people develop rapid food absorption and strong hormone signals that trigger extra insulin release and low blood sugar after meals. Medical News Today+1

7. Drug-induced hyperinsulinism – occurs when medicines such as sulfonylureas or meglitinides stimulate the pancreas to release too much insulin, or when too much injected insulin is given. Healthline+1

8. Insulin-resistance–related hyperinsulinemia – in obesity, metabolic syndrome, and type 2 diabetes risk, the body does not respond well to insulin, so the pancreas makes extra insulin for many years. NCBI+1

9. Autoimmune insulin syndrome – rare condition where antibodies bind to insulin, causing unstable insulin levels and episodes of low blood sugar. Medical News Today+1

10. Hyperinsulinism in infants of diabetic mothers – babies born to mothers with diabetes may have high insulin levels before birth and hypoglycemia soon after birth. NCBI+1

Causes of Hyperinsulinism

1. Genetic mutations in KATP channel genes (ABCC8, KCNJ11)
   Changes in these genes affect a channel on beta cells that helps control insulin release. When the channel does not work properly, beta cells stay “on” and keep releasing insulin even when blood sugar is low, causing congenital hyperinsulinism. PMC+1

2. Other monogenic causes (GLUD1, GCK, HADH and others)
   Mutations in several other genes can change how beta cells sense glucose or work with energy, leading to constant insulin release. Each gene affects a different part of the signaling pathway, but the result is the same: too much insulin and hypoglycemia. PMC+1

3. Diffuse beta-cell hyperplasia (nesidioblastosis)
   In some children, many beta cells are enlarged and overactive. The pancreas looks “normal-sized” but the cells keep secreting insulin inappropriately. This diffuse form often needs strong medicines and sometimes partial removal of the pancreas. Physiology Journals+1

4. Focal adenomatous hyperplasia in the pancreas
   In focal congenital hyperinsulinism, a small patch of beta cells grows abnormally and makes too much insulin. If this focus can be found on imaging, localized surgery can cure the child by removing just that small part. PMC+1

5. Insulinoma (insulin-secreting tumor)
   An insulinoma is usually a small, benign tumor made of beta cells. It releases insulin without control, often causing fasting hypoglycemia in adults. Symptoms often improve when the tumor is removed. ScienceDirect+1

6. Perinatal stress (birth asphyxia, poor growth in the womb)
   Babies who suffer low oxygen, infection, or poor growth before or around birth can develop temporary hyperinsulinism. The stressed beta cells become more active and keep releasing insulin, causing low blood sugar in the first days or weeks of life. NCBI+1

7. Infants of diabetic mothers
   If the mother’s blood sugar is high during pregnancy, extra glucose crosses the placenta to the baby. The baby’s pancreas responds by making a lot of insulin. After birth, when the sugar supply suddenly drops, the baby’s insulin is still high, and hypoglycemia can occur. NCBI+1

8. Beckwith–Wiedemann and other overgrowth syndromes
   Some genetic overgrowth conditions, such as Beckwith–Wiedemann syndrome, include hyperinsulinism as a feature. These children may have large birth weight, enlarged organs, and frequent severe hypoglycemia due to excess insulin. Frontiers+1

9. Metabolic syndrome and obesity
   In older children, adolescents, and adults, excess body fat can cause insulin resistance. To keep blood sugar in the normal range, the pancreas makes more insulin. This chronic hyperinsulinemia can exist for years before diabetes appears. NCBI+1

10. Sedentary lifestyle and high-calorie diet
    Low physical activity and diets high in sugar, refined carbohydrates, and unhealthy fats can worsen insulin resistance. The body then needs higher insulin levels to handle the same meal, leading to a state of chronic hyperinsulinemia. NCBI+1

11. Polycystic ovary syndrome (PCOS)
    Many people with PCOS have insulin resistance and compensatory hyperinsulinemia. The high insulin levels can worsen hormonal imbalance, increase androgen levels, and lead to weight gain, making the condition a cycle. NCBI+1

12. Type 2 diabetes risk and prediabetes
    Before type 2 diabetes develops, insulin resistance builds over years. During this time, the pancreas increases insulin output to keep glucose normal. This stage is characterized by high insulin levels, even if blood sugar is still within range. NCBI+1

13. Nonalcoholic fatty liver disease (NAFLD)
    Extra fat in the liver is linked with insulin resistance and higher insulin levels. The liver does not respond properly to insulin, continues making glucose, and the pancreas compensates by secreting more insulin to control blood sugar. NCBI+1

14. Medicines that stimulate insulin release
    Some diabetes medicines like sulfonylureas and meglitinides trigger the pancreas to release more insulin. Overdose, accidental use, or use by someone without diabetes can cause dangerous hyperinsulinemic hypoglycemia. Medical News Today+1

15. Excess injected insulin
    Too high a dose of injectable insulin can cause the insulin level in the blood to rise far above need. This may be accidental (dose error) or due to wrong timing with meals, leading to very low blood sugar. Wikipedia+1

16. Autoimmune insulin syndrome
    In this rare condition, the body makes antibodies against insulin. These antibodies can hold insulin and then suddenly release it, causing large swings in insulin activity and repeated episodes of hypoglycemia with measured high insulin levels. Medical News Today+1

17. Insulin receptor or signaling problems
    Some rare disorders affect the insulin receptor or signaling pathways. Tissues do not respond well, so the pancreas increases insulin release. This can cause both features of insulin resistance and periods of hypoglycemia due to very high insulin. NCBI+1

18. Chronic kidney disease
    The kidneys help remove insulin from the blood. When kidney function drops, insulin can stay in the circulation longer, leading to higher effective insulin levels and episodes of hypoglycemia, especially if insulin or secretagogue drugs are used. Healthline+1

19. Hormonal disorders (Cushing’s, acromegaly, etc.)
    Conditions with excess cortisol or growth hormone can cause insulin resistance. The pancreas then secretes more insulin to overcome this, leading to hyperinsulinemia; later, blood sugar may also rise. NCBI+1

20. After bariatric or gastric surgery
    Some people develop “late dumping” or post-bariatric hypoglycemia. Food moves quickly into the intestine, causing a strong hormone (incretin) response and a big insulin surge after meals, sometimes leading to low blood sugar. berkeleymedical.com.au+1

Symptoms of Hyperinsulinism (15 Symptoms, Simple English)

1. Shakiness and tremor
   When blood sugar drops, the body releases stress hormones like adrenaline. This can cause the hands or body to shake, making it hard to hold objects or write. Medscape+1

2. Sweating and feeling clammy
   People with low blood sugar often sweat suddenly, even in a cool room. The skin may feel cold and damp. This is another sign that the body is under stress from hypoglycemia. Medscape+1

3. Hunger and food cravings
   The brain senses that blood sugar is falling and sends strong hunger signals. People may feel they must eat quickly, often craving sugary foods to raise their blood sugar. Cleveland Clinic+1

4. Headache
   When the brain does not get enough glucose, headaches can occur. The pain can be mild or severe and may come with dizziness or blurred vision. Medscape+1

5. Dizziness or light-headed feeling
   Low blood sugar can make a person feel as if they might faint, especially when standing up or moving quickly. This can be dangerous if they are driving or operating machines. Medscape+1

6. Confusion and difficulty thinking
   Glucose is the main fuel for the brain. When levels fall, people may think slowly, answer questions wrongly, or act strangely. They may have trouble concentrating, speaking clearly, or solving simple tasks. Medscape+1

7. Behavior changes, irritability, or unusual sleepiness
   Children with hyperinsulinism may become irritable, fussy, or unusually quiet. Babies may be hard to wake, cry weakly, or seem “not themselves.” Adults may feel very tired or sleepy. rarediseases.org+1

8. Poor feeding in infants
   Babies with congenital hyperinsulinism may feed poorly, stop sucking, or refuse the breast or bottle when their blood sugar is low. They may sweat, look pale, or become floppy. rarediseases.org+1

9. Floppiness or low muscle tone (hypotonia)
   Some infants may feel “floppy” when held. Their arms and legs may hang loosely because the muscles are not getting enough energy from glucose. Brieflands+1

10. Seizures (fits, convulsions)
    Very low blood sugar can trigger seizures in infants, children, and adults. The person may stiffen, jerk, roll their eyes, or stop responding. Seizures need emergency care and are a strong sign that the brain is being injured by hypoglycemia. Brieflands+1

11. Loss of consciousness or fainting
    In severe cases, a person with hyperinsulinemic hypoglycemia can lose consciousness. They may look pale, sweat, and become unresponsive, which is a medical emergency. Medscape+1

12. Fast heartbeat (palpitations)
    The heart beats faster when adrenaline is released during low blood sugar. People may feel their heart racing or pounding in their chest or neck. Medscape+1

13. Blurred or double vision
    The eyes and brain both need glucose. When blood sugar is low, vision can become blurry, or people may see double, making it hard to read or walk safely. Medscape+1

14. Weight gain and difficulty losing weight (in chronic hyperinsulinemia)
    High insulin levels encourage the body to store fat and stop breaking it down. People with insulin-resistance–related hyperinsulinemia often gain weight easily and find it hard to lose weight even when they try. Cleveland Clinic+1

15. Long-term learning or developmental problems (in children)
    Repeated episodes of low blood sugar during early life can hurt the developing brain. Some children with poorly controlled congenital hyperinsulinism may later have delays in walking, talking, or learning. Frontiers+1

Important: These symptoms can be serious. Anyone with repeated low blood sugar, seizures, or loss of consciousness needs urgent medical care. This description is for education only and is not a substitute for seeing a doctor.

Diagnostic Tests for Hyperinsulinism

Doctors use a mix of physical examination, bedside/manual tests, laboratory and pathological tests, electrodiagnostic tests, and imaging tests to diagnose hyperinsulinism and find its cause. Karger Publishers+1

Physical Examination (Basic Bedside Checks)

1. Full physical examination (vital signs, growth, body build)
The doctor checks height, weight, body mass index (BMI), head size in babies, blood pressure, and heart rate. In infants, they look for large birth weight, big tongue, or other features of syndromes. In older children and adults, they look for signs of obesity or metabolic syndrome. These clues help decide whether hyperinsulinism is congenital, syndromic, or related to insulin resistance. Frontiers+1

2. Neurologic examination
The doctor checks muscle tone, reflexes, movement, and mental status. In infants, they look at how the baby responds, holds the head, and moves arms and legs. In older patients, they test strength, coordination, and thinking. Abnormal findings may show that repeated hypoglycemia has affected the brain. Brieflands+1

3. Abdominal examination
The abdomen is examined for enlarged liver or kidneys and other organ changes. In some syndromic forms, organs can be larger than normal. In adults with insulinoma, the abdomen usually feels normal, but exam can still help detect other possible causes of symptoms. rarediseases.org+1

4. Skin examination (including acanthosis nigricans and injection sites)
The doctor looks for dark, velvety patches of skin, especially on the neck and underarms, which is called acanthosis nigricans and is linked to insulin resistance and high insulin levels. They may also look for lipodystrophy (fat loss or lumps) at insulin injection sites, which can affect insulin absorption and blood sugar levels. NCBI+1

Manual and Bedside Tests

5. Capillary blood glucose (finger-prick test)
This quick test measures sugar in a drop of blood from the finger or heel. It is used when symptoms appear to confirm hypoglycemia. If the reading is low, further testing is done to understand whether insulin is too high at the same time. NCBI+1

6. Critical sample during a hypoglycemia episode
When blood sugar is confirmed low, doctors draw extra blood (“critical sample”) to measure insulin, C-peptide, ketones, and other hormones. If insulin is inappropriately high when glucose is low, hyperinsulinism is very likely. This sample is essential for diagnosis. e-apem.org+1

7. Supervised fasting test
In older children and adults, a carefully monitored fast in the hospital can help show how the body regulates blood sugar. Blood is taken at intervals. If blood sugar drops and insulin stays high or undetectably low ketones are seen, hyperinsulinism or insulinoma may be present. This test must be done under expert supervision because it can be risky. Medscape+1

8. Mixed-meal or provocation test
Some people have low blood sugar mainly after eating, especially after carbohydrate-rich meals or after bariatric surgery. A mixed-meal test uses a standardized meal, and blood samples are taken over a few hours to see how glucose and insulin levels change. An exaggerated insulin response with later hypoglycemia suggests post-prandial hyperinsulinism. Medical News Today+1

Laboratory and Pathological Tests

9. Plasma insulin level during hypoglycemia
A key test is measuring insulin in the blood at the same time as low glucose. In normal people with low sugar, insulin should be very low or undetectable. If insulin is still measurable or high when glucose is low, it indicates hyperinsulinism or exogenous insulin use. e-apem.org+1

10. C-peptide and proinsulin levels
C-peptide is released in equal amounts with natural insulin from the pancreas, while injected insulin does not contain C-peptide. Measuring C-peptide helps distinguish between too much natural insulin (endogenous hyperinsulinism) and injected insulin. High proinsulin may be seen in insulinoma and some congenital forms. Physiology Journals+1

11. Beta-hydroxybutyrate and free fatty acids
In normal fasting, ketones and free fatty acids rise as the body starts burning fat. In hyperinsulinism, insulin blocks fat breakdown, so beta-hydroxybutyrate and free fatty acids stay low even during hypoglycemia. This pattern strongly suggests excess insulin action. e-apem.org+1

12. Counter-regulatory hormones (cortisol, growth hormone)
When blood sugar is low, the body should release cortisol and growth hormone to help raise glucose. Measuring these during hypoglycemia helps rule out other causes such as adrenal insufficiency or growth hormone deficiency, and confirms that the defense system is working. If these are normal or high but hypoglycemia persists with high insulin, hyperinsulinism is more likely. NCBI+1

13. Sulfonylurea and drug screen
A blood or urine test can look for sulfonylureas and similar medicines that stimulate insulin secretion. This is important to rule out accidental, purposeful, or hidden use of these drugs as a cause of hyperinsulinemic hypoglycemia. Healthline+1

14. Insulin autoantibody testing
In suspected autoimmune insulin syndrome, blood tests can look for antibodies that bind insulin. A positive test, along with high insulin and C-peptide during hypoglycemia, supports this rare diagnosis. MDPI+1

15. Genetic testing for congenital hyperinsulinism
A blood sample can be sent for genetic analysis of genes known to cause congenital hyperinsulinism, such as ABCC8, KCNJ11, GLUD1, GCK, and others. Finding a mutation can confirm the diagnosis, help decide treatment (for example, whether diazoxide will work), and guide family counseling. PMC+1

Electrodiagnostic Tests

16. Electroencephalogram (EEG)
EEG records the electrical activity of the brain. In children who have seizures due to hypoglycemia, EEG can help confirm seizure activity, look for patterns of brain injury, and guide long-term neurological care. It does not diagnose hyperinsulinism directly, but it shows the effect of repeated low blood sugar on the brain. Brieflands+1

17. Electrocardiogram (ECG)
An ECG records the heart’s electrical activity. Severe hypoglycemia and some medicines used to treat hyperinsulinism can affect heart rhythm. An ECG helps detect abnormal rhythms, prolonged QT interval, or other heart stress during hypoglycemia episodes, adding to the safety of treatment. Karger Publishers+1

Imaging Tests

18. Abdominal ultrasound
Ultrasound uses sound waves to create pictures of the organs. It can show liver size, kidney structure, and sometimes big tumors. In hyperinsulinism, ultrasound may help detect syndromic organ enlargements or large pancreatic masses, although small insulinomas are often too tiny to see. rarediseases.org+1

19. CT or MRI scan of the pancreas
Computed tomography (CT) and magnetic resonance imaging (MRI) give detailed images of the pancreas and nearby organs. These scans help in finding insulinomas or structural abnormalities in adults and sometimes in children. They are important for planning surgery if a tumor is found. ScienceDirect+1

20. 18F-DOPA PET/CT or other specialized PET scans
In many specialized centers, a type of PET scan using 18F-DOPA or other tracers is used to find focal areas of beta-cell overgrowth in congenital hyperinsulinism. These scans can show a “hot spot” in the pancreas that produces extra insulin and can guide surgeons to remove only that portion, preserving as much normal pancreas as possible. Frontiers+1

Non-pharmacological treatments

1. Frequent small meals
Eating small meals and snacks every 2–3 hours can help keep blood sugar steady. The goal is to avoid long gaps without food, because fasting allows insulin to push sugar too low. Regular meals with complex carbohydrates, a little protein, and healthy fat give a slow, steady release of sugar into the blood and can reduce sudden dips. This is often the first daily strategy used at home. Congenital Hyperinsulinism International+1

2. Night-time feeds or continuous feeds
Some babies and children with hyperinsulinism cannot safely go many hours overnight without food. Doctors may recommend night-time bottle feeds or continuous feeds through a tube using a pump. This slowly gives glucose or formula all night and helps prevent dangerous low sugar episodes during sleep. Parents are taught how to manage pumps and what to do if feeds stop unexpectedly. Children’s Hospital of Philadelphia+1

3. Avoiding prolonged fasting
A key lifestyle rule is “no long fasting.” The safe fasting time is different for each child and is tested in hospital. At home, caregivers follow clear instructions about maximum hours without food. During illness, the fasting time is often shortened because children eat less and are at higher risk of hypoglycemia. Written “sick-day rules” help families act early. Karger Publishers+1

4. Dextrose gel or glucose gel during mild lows
Some centers recommend a special glucose gel rubbed inside the cheek at the start of a mild low blood sugar episode, along with oral feeding. The gel gives quick sugar that is absorbed through the mouth lining. It is not a replacement for medical care, but it can be part of an emergency plan while arranging help. SAGE Journals+1

5. Home blood glucose monitoring
Regular finger-prick blood sugar checks at home, or sometimes continuous glucose monitoring (CGM), help families see patterns and catch lows early. Results guide when to feed, when to give extra carbohydrates, and when to seek urgent help. Monitoring is especially important after changing medicines, during illness, or after surgery. Dove Medical Press+1

6. Emergency hypoglycemia action plan
Families usually receive a written emergency plan from the specialist team. It explains what to do when blood sugar falls below certain numbers, which fluids or carbohydrates to give, and when to go to the emergency department. This plan reduces panic and helps all caregivers act quickly and consistently to protect the brain. Karger Publishers+1

7. Medical ID bracelet or card
A medical bracelet, necklace, or card saying “Hyperinsulinism – risk of severe hypoglycemia” helps emergency staff immediately understand the condition. If a child becomes confused, drowsy, or has a seizure outside home, responders can treat low blood sugar fast even before parents arrive. This simple step may prevent brain injury.

8. Dietitian-guided carbohydrate planning
A pediatric dietitian helps design a meal plan with the right amount and type of carbohydrates. They often include slow-release carbs like whole grains and sometimes uncooked cornstarch at night, depending on age and local practice. The aim is to match food to the child’s insulin pattern and reduce swings in blood sugar. Congenital Hyperinsulinism International+1

9. Cornstarch or slow-release starch (case-by-case)
In some children, especially older ones, uncooked cornstarch or special slow-release starch may be used at bedtime. It breaks down slowly in the gut and provides a long, gentle supply of glucose during the night. It must only be used under specialist guidance, because dosing is individual and safety must be checked. Congenital Hyperinsulinism International+1

10. School or daycare care plan
Children with hyperinsulinism need support at school. A written plan tells staff when to offer snacks, how to recognize low blood sugar, how to check glucose (if allowed), and when to call parents or emergency services. Training teachers reduces delays in treatment and helps the child join normal activities more safely. Frontiers+1

11. Structured physical activity with planning
Exercise is healthy but can lower blood sugar. Families are taught to check sugar before and after vigorous activity, to give an extra snack if needed, and to avoid intense activity when sugar is already low. Over time, they learn which types of play are safe and how to prepare. This keeps the child active but protected.

12. Illness and “sick-day” feeding rules
During fever, vomiting, or diarrhea, children may not eat well but insulin can still be high. Sick-day rules often advise more frequent glucose checks, extra sugary drinks or oral rehydration solution, and a lower threshold for coming to hospital for IV glucose. This structured response reduces risk of sudden, severe hypoglycemia. Karger Publishers+1

13. Psychological and family support
Caring for a child with hyperinsulinism is stressful. Sleep is disturbed by night checks and feeds. Parents may fear brain damage from every low. Counseling, support groups, or social work support help families cope with anxiety, learn problem-solving skills, and avoid burnout. A resilient family makes day-to-day care safer for the child. Frontiers+1

14. Multidisciplinary team follow-up
Best care usually comes from a team that includes endocrinologists, nurses, dietitians, surgeons, psychologists, and sometimes geneticists. Regular follow-up visits review growth, development, glucose records, medicines, and nutrition. The team adjusts treatment as the child grows or as the type of hyperinsulinism becomes clearer. Frontiers+1

15. Genetic counseling for families
Many forms of congenital hyperinsulinism are genetic. Genetic testing and counseling help parents understand inheritance patterns, future pregnancy risks, and the chance of hyperinsulinism in siblings. This supports informed family planning and may guide decisions about prenatal testing in some families. Frontiers+1

16. Education about early symptoms of low sugar
Parents, older children, and caregivers are taught to notice early warning signs such as sweating, shakiness, fast heartbeat, irritability, or sudden sleepiness. Quick recognition allows faster feeding or glucose treatment, often preventing seizures or hospital visits. Repeated practice and simple language make the education more effective. Karger Publishers+1

17. Safe storage of glucose and emergency supplies
Families are advised to keep glucose gel, fast-acting sugary drinks, and carb snacks in several places, such as at home, in school bags, and in the car. Having supplies within reach every time reduces delays when a low occurs. Checking expiry dates and replacing items regularly is part of routine home care.

18. Use of continuous glucose monitoring (where available)
In some settings, continuous glucose monitors (CGMs) are used in children with difficult-to-control hyperinsulinism. They give near-real-time readings and alarms for lows. CGMs do not replace finger-pricks, but they can help families see trends and may reduce the time spent in undetected hypoglycemia. Dove Medical Press+1

19. Hospital-based fasting and safety testing
Before changing feeds or medicines, teams may do controlled fasting tests in hospital. Blood sugar, ketones, and insulin are checked while the child is carefully observed. These tests help decide how long the child can safely fast and which treatments are working. They should never be done at home. Karger Publishers+1

20. Clear transition planning to adult services
Older teenagers with ongoing hyperinsulinism need a planned transition to adult endocrine services. This includes teaching them to manage their own glucose checks, medicines, and appointments. Good transition planning lowers the risk that care will be lost when the teen becomes an adult. Frontiers+1

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Drug treatments

Important: All medicines for hyperinsulinism must be chosen and dosed only by specialists. Doses, schedules, and combinations are very individual, especially in babies and children. Never change or start medicines on your own.

1. Diazoxide (PROGLYCEM and similar products)
Diazoxide is usually the first-line drug for many types of congenital hyperinsulinism. It reduces insulin release from pancreatic beta cells and raises blood sugar. It is an oral medicine used for symptomatic hypoglycemia caused by hyperinsulinism. Typical dosing is based on body weight and carefully adjusted in hospital. Common side effects include fluid retention, heart failure in some infants, body hair growth, and nausea, so close monitoring is needed. FDA Access Data+2FDA Access Data+2

2. Octreotide (SANDOSTATIN and related brands)
Octreotide is a somatostatin analogue. It blocks release of several hormones, including insulin, and can help control blood sugar when diazoxide fails or is not tolerated. It is given as injections under the skin or as an infusion. Doses are titrated slowly. Side effects include stomach upset, gallstones, and changes in blood sugar (sometimes high or low). FDA Access Data+2FDA Access Data+2

3. Long-acting octreotide (SANDOSTATIN LAR DEPOT)
A long-acting injectable form of octreotide can provide more stable suppression of insulin over weeks instead of hours. It is usually used in older children or adults when frequent injections are difficult. It is injected into muscle every few weeks by a health professional. Risks are similar to short-acting octreotide, including gallbladder problems and digestive issues. FDA Access Data+1

4. Lanreotide (SOMATULINE DEPOT and generic lanreotide injection)
Lanreotide is another long-acting somatostatin analogue. It is approved for acromegaly and certain neuroendocrine tumors, but has been used off-label in some hyperinsulinism cases. It is given by deep subcutaneous injection every few weeks. It can reduce both insulin and glucagon secretion, so sugar must be monitored closely. Side effects include gallstones, digestive upset, and possible changes in glucose control. FDA Access Data+2FDA Access Data+2

5. Nifedipine
Nifedipine is a calcium-channel blocker usually used for high blood pressure. In rare cases of congenital hyperinsulinism, it has been reported to help reduce insulin release from beta cells by blocking calcium entry into the cells. Evidence is limited and mixed, so it is usually considered only when standard drugs fail. Side effects include low blood pressure, flushing, and headache. jcrpe.org+1

6. Glucagon (injection or infusion)
Glucagon is a hormone that tells the liver to release stored glucose. In hyperinsulinism, intravenous or subcutaneous glucagon can be used in emergencies or as a continuous infusion to stop severe hypoglycemia while other treatments are arranged. Side effects may include nausea, vomiting, and, rarely, allergic reactions. It is a rescue medicine, not a long-term single solution. Karger Publishers+1

7. Intravenous dextrose (hospital use)
In hospital, the main acute treatment for severe hypoglycemia is intravenous dextrose solution. A bolus is given to quickly raise blood sugar, followed by a continuous infusion adapted to keep glucose above a safe level. This is a life-saving emergency medicine and must be managed with careful monitoring and infusion pumps. Karger Publishers+1

8. Sirolimus
Sirolimus is an mTOR inhibitor originally used as an immunosuppressant. It has been tried in severe hyperinsulinism that does not respond to diazoxide or octreotide, aiming to reduce abnormal beta-cell activity. Some small studies show benefit, but there are serious risks such as infections, high lipids, and possible long-term side effects, so many experts advise extreme caution and limit use to research settings. PMC+2ScienceOpen+2

9. Everolimus
Everolimus is another mTOR inhibitor used for certain cancers and as an immunosuppressant. Case reports show that it can help control refractory hypoglycemia in malignant insulinoma and some hyperinsulinism situations by reducing insulin secretion and tumor growth. It has many potential side effects, including mouth ulcers, infections, and changes in blood counts, so its use is highly specialist and usually off-label. New England Journal of Medicine+2PMC+2

10. Glucagon-like peptide-1 (GLP-1) receptor pathway drugs (experimental)
Some researchers are exploring GLP-1 pathway drugs, often used for diabetes or obesity, to understand their effect on insulin secretion and hypoglycemia. In general, GLP-1 agonists increase insulin, so they are not standard treatments for hyperinsulinism. However, careful use in complex endocrine tumors may be studied. At present, they are not routine therapy for congenital hyperinsulinism. OUP Academic+1

11. Hydrochlorothiazide combinations with diazoxide (in some regimens)
Because diazoxide can cause fluid retention, diuretics such as hydrochlorothiazide are sometimes added in small doses in older children to manage swelling and high blood pressure. This is not to treat hyperinsulinism directly but to control diazoxide side effects. Dose and duration must be carefully balanced to avoid dehydration or electrolyte problems. FDA Access Data+1

12. Continuous subcutaneous insulin antagonism approaches (research)
Some research protocols look at combining somatostatin analogues, glucagon, and controlled dextrose to “antagonize” the effect of too much insulin. These are complex regimens, carefully managed in specialist centers, and are not self-managed therapies. The overall goal is to stabilize glucose while avoiding extremely high insulin levels. Dove Medical Press+1

13. Second-generation somatostatin analogues (e.g., pasireotide – experimental)
Pasireotide has stronger binding to some somatostatin receptors than octreotide. Early case reports suggest it might help some children whose hyperinsulinism does not respond to first-generation analogues, but experience is limited and side effects such as hyperglycemia and adrenal effects are concerns. Its use remains experimental in this setting. SAGE Journals+1

14. Other calcium-channel blockers (research use)
Other calcium-channel blockers beyond nifedipine have been tested in laboratory and case reports to see if they can reduce insulin secretion. Data are sparse, and these drugs may cause low blood pressure or heart rhythm changes. They are not standard care and are only used in special situations under expert oversight. jcrpe.org+1

15. Corticosteroids (very limited, special situations)
Steroids such as hydrocortisone can raise blood sugar by increasing glucose production and reducing insulin sensitivity. In some acute, complex endocrine conditions they may be used, but they are not a main long-term treatment for hyperinsulinism because of many side effects, including weight gain, high blood pressure, and growth problems in children. Medscape+1

16. Beta-blockers to support heart function (supportive, not direct treatment)
When diazoxide causes fluid retention and strain on the heart, cardiologists sometimes use medicines such as beta-blockers or ACE inhibitors to support heart function. These drugs do not treat insulin directly but help manage complications of therapy. They require heart monitoring and specialist follow-up. FDA Access Data+1

17. Proton pump inhibitors or H2 blockers (for gastric side effects)
Children on long-term somatostatin analogues or mTOR inhibitors may develop gastrointestinal side effects like reflux, nausea, or ulcers. Acid-reducing drugs may be used to protect the stomach. They do not treat hyperinsulinism itself but make long-term treatment more tolerable. FDA Access Data+1

18. Antibiotics and infection prophylaxis with mTOR inhibitors
Because sirolimus and everolimus weaken immune responses, some protocols include preventive antibiotics or careful infection monitoring. This is supportive care, not treatment of hyperinsulinism, but it is crucial to keep patients safe while on these powerful medicines. ScienceOpen+1

19. Anti-diarrheal and gallbladder-supportive medicines
Somatostatin analogues can cause diarrhea, fat malabsorption, and gallstones. Doctors may use bile-acid drugs or pain medicines to manage these side effects. Again, these do not treat insulin directly but help patients stay on necessary therapy. FDA Access Data+1

20. Clinical-trial medicines (investigational drugs)
Some children with severe, treatment-resistant hyperinsulinism are enrolled in clinical trials testing new drugs or new combinations, such as novel mTOR-pathway drugs or gene-targeted therapies. These medicines are experimental, closely monitored, and only available in specialized research centers. ClinicalTrials.gov+1

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Dietary molecular supplements

Note: Evidence for supplements in congenital hyperinsulinism is limited. Most data come from studies in insulin resistance or diabetes. Supplements can interact with medicines or worsen hypoglycemia. Always ask the care team before using any of them.

1. Omega-3 fatty acids (fish oil) – May support heart health and reduce inflammation, which is helpful for long-term metabolic health. Omega-3s do not fix hyperinsulinism but may support general wellbeing in older patients at risk of high lipids from mTOR inhibitors or other drugs.

2. Vitamin D – Important for bone health and immune function. Children with chronic illness, limited sun exposure, or certain medicines may have low vitamin D. Correcting deficiency supports growth and bone strength but does not directly cure hyperinsulinism.

3. Magnesium – Magnesium helps many enzymes involved in glucose use and insulin signaling. In people with insulin resistance, low magnesium can worsen control. In hyperinsulinism, it may be corrected if low but is not a primary therapy. High doses can cause diarrhea and should be supervised.

4. Chromium – Chromium is sometimes marketed for “blood sugar support” because it is involved in insulin action. Evidence in children is weak, and in congenital hyperinsulinism the issue is too much insulin, not insulin resistance. Use is generally not routine and should be avoided without specialist advice.

5. Alpha-lipoic acid – An antioxidant studied mainly in diabetic nerve damage. It may have mild effects on insulin sensitivity. It is not standard in hyperinsulinism and can have side effects like nausea and low blood sugar, so it should not be used without medical supervision.

6. Inositol (myo-inositol, D-chiro-inositol) – These molecules help transmit insulin signals inside cells. They are used mostly in polycystic ovary syndrome and insulin resistance. In hyperinsulinism, they are not well studied and could theoretically worsen lows, so any use must be cautious.

7. Coenzyme Q10 – A mitochondrial cofactor with antioxidant properties. It is sometimes used to support muscle and heart energy metabolism. It does not directly change insulin levels but may support general health in people on long-term heavy medicines.

8. Curcumin (from turmeric) – A plant compound with anti-inflammatory and antioxidant effects studied in metabolic syndrome. It can interact with many drugs and may affect liver enzymes, so it should not be used without checking with doctors, especially in children.

9. Fiber supplements (psyllium, resistant starch) – Extra fiber slows absorption of carbohydrates and can help smooth blood sugar swings in some settings. In hyperinsulinism, dietitians may use high-fiber foods carefully to avoid large spikes and dips in glucose, but they still prioritize overall calorie and glucose needs.

10. Probiotics – These support gut bacteria balance and may have small effects on metabolism and immunity. They do not treat hyperinsulinism but may help with digestion, especially in children on multiple medicines. Choices should be age-appropriate and discussed with the care team.

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Immunity-booster and regenerative / stem-cell-related drugs

There are no approved stem-cell or “immunity booster” drugs that cure hyperinsulinism. The options below are mostly experimental or supportive and are mentioned for information only.

1. Sirolimus as an immunosuppressant with metabolic effect – Sirolimus suppresses immune activity and can reduce abnormal beta-cell growth and insulin secretion in some severe cases. Because it weakens immunity and has many long-term risks, experts recommend using it only when standard treatments have failed and usually in research settings. PMC+2ScienceOpen+2

2. Everolimus in tumor-related hyperinsulinism – In malignant insulinoma, everolimus can shrink tumors and directly reduce insulin secretion, improving hypoglycemia. It also strongly affects immunity and has cancer-drug side effects, so it is used only under oncology and endocrine supervision. PMC+2ScienceDirect+2

3. Hematopoietic stem-cell transplantation (very rare, research) – Stem-cell or bone-marrow transplantation is not a standard treatment for hyperinsulinism. Theoretically, gene-corrected stem cells might be used in future gene therapies, but right now such approaches are still experimental and mainly discussed in research, not routine clinical practice. Frontiers

4. Islet cell transplantation (opposite indication – for diabetes) – Islet transplantation is used in some adults with type 1 diabetes to restore insulin production. In hyperinsulinism, where insulin is already excessive, this approach is not appropriate. It is mentioned only to explain that current transplantation therapies target low-insulin diseases, not high-insulin ones. Frontiers+1

5. Gene-targeted therapies in development
   Future treatments may try to correct specific genetic changes (such as KATP channel mutations) using gene-editing or RNA-based technologies. These ideas are still in preclinical or early research stages and are not available as routine therapy. Safety and long-term effects must be carefully studied first. Frontiers+1

6. Vaccinations and routine immune support
   The most realistic “immunity booster” for children with hyperinsulinism is simply staying up-to-date with routine vaccinations, getting flu shots, and seeking early care for infections. This protects fragile children, especially those with central lines or those taking mTOR inhibitors that weaken the immune system. ScienceOpen+1

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Surgeries and procedures

1. Focal lesionectomy (removal of a focal pancreatic lesion)
   In focal congenital hyperinsulinism, only a small part of the pancreas is abnormal. Surgeons can remove just that focal area. If successful, this can cure the hyperinsulinism while keeping most of the pancreas intact. It requires special imaging and experienced centers to locate the lesion correctly. Semantic Scholar+1

2. Near-total pancreatectomy
   In diffuse disease, almost the whole pancreas is affected. When medicines and diet cannot control hypoglycemia, surgeons may remove 95–98% of the pancreas. This can greatly reduce episodes of low blood sugar but may later cause diabetes and digestion problems. Families must understand these long-term risks before surgery. Semantic Scholar+1

3. Laparoscopic (keyhole) partial pancreatectomy
   Some centers perform parts of the surgery with minimally invasive “keyhole” techniques. The goal is the same—removing abnormal tissue—but with smaller incisions and possibly faster recovery. The choice between open and laparoscopic surgery depends on the child’s anatomy and the surgeon’s experience. Semantic Scholar+1

4. Gastrostomy tube (feeding tube) placement
   A feeding tube placed through the skin into the stomach (G-tube) provides a secure route for continuous glucose or formula feeds at home. It helps children who cannot maintain blood sugar through mouth feeding alone. The procedure is usually done under anesthesia and needs ongoing care to prevent infection or dislodgement. Children’s Hospital of Philadelphia+2Congenital Hyperinsulinism International+2

5. Central venous line or port placement
   Some children need long-term intravenous glucose or frequent medicines. A central venous catheter or implanted port allows reliable access to large veins. It is placed in a short surgical procedure. It makes therapy easier but carries risks like infection and blood clots, so careful line care is essential. Dove Medical Press+1

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Prevention tips

1. Follow the feeding and fasting plan exactly as given by the specialist team.

2. Keep regular clinic visits so medicines and diet can be adjusted as the child grows.

3. Check blood sugar as often as recommended and keep a written or digital log.

4. Act early at the first sign of low sugar—do not wait for severe symptoms.

5. Teach all caregivers (family, babysitters, teachers) about the condition and emergency steps.

6. Keep glucose gel, sugary drinks, and snacks with you whenever the child leaves home.

7. Use written sick-day rules during illness and seek help early if the child is not eating.

8. Keep up-to-date with vaccines to reduce infection-related hospital stays.

9. Store medicines and feeds correctly and check expiry dates regularly.

10. Ask for psychological and social support to manage stress so that care remains consistent and safe. Frontiers+2SpringerLink+2

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When to see doctors or go to emergency care

You should contact your doctor urgently or go to emergency care if:

• Blood sugar remains low despite giving extra carbohydrate or glucose as instructed.

• The child becomes very sleepy, confused, irritable, or cannot be woken easily.

• There are seizures, jerking movements, or unusual staring spells.

• The child is vomiting repeatedly and cannot keep food or drink down.

• You think the feeding tube, pump, or IV line is blocked, leaking, or has stopped.

• There is a fever with redness or pus around a central line or G-tube site.

• Breathing becomes fast or labored, or the child looks very unwell or pale.

For routine follow-up, you should also see the doctor if:

• Lows are happening more often than before.

• Growth, weight, or development seems to slow down.

• New medicines or supplements are being considered.

Early review helps prevent serious complications from repeated hypoglycemia. Karger Publishers+2Frontiers+2

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What to eat and what to avoid

1. Eat regular meals with complex carbs (whole grains, brown rice, oats, lentils) to provide slow, steady energy.

2. Eat some protein (egg, dairy, lean meat, fish, legumes) at every meal or snack to help stabilize blood sugar.

3. Eat healthy fats (olive oil, nuts if age-safe, avocado) as advised, to give extra calories and sustained energy.

4. Eat nutrient-rich fruits and vegetables in controlled portions, pairing them with protein or fat to avoid rapid sugar changes.

5. Eat snacks before long car journeys, activities, or medical visits, according to your team’s guidance.

6. Avoid long gaps without food; do not skip meals or snacks.

7. Avoid drinks with very large amounts of simple sugar unless being used as part of a planned treatment for a low; they can cause sharp ups and downs in blood sugar.

8. Avoid very low-carbohydrate fad diets, which are unsafe for children with hyperinsulinism.

9. Avoid new supplements, herbal products, or weight-loss products without medical advice.

10. Avoid high-caffeine energy drinks, which may mask hypoglycemia symptoms and upset the heart in children on certain medicines. Congenital Hyperinsulinism International+1

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Frequently asked questions

1. Is hyperinsulinism the same as diabetes?
No. In most diabetes, insulin is too low or does not work properly, causing high blood sugar. In hyperinsulinism, the pancreas makes too much insulin, driving blood sugar too low. Treatments and risks are very different. Frontiers+1

2. Can hyperinsulinism be cured?
Some forms, especially focal congenital hyperinsulinism, can be cured by surgery that removes the abnormal part of the pancreas. Diffuse forms may improve with age but often need long-term medical and dietary management. Each case is different, so prognosis must be discussed with the specialist team. Semantic Scholar+1

3. Will my child definitely get brain damage from low sugar?
Not always. The risk depends on how low the sugar goes, how often it happens, and how quickly it is treated. Prompt treatment, regular monitoring, and good long-term control greatly reduce the chance of permanent brain injury. Frontiers+1

4. Why is diazoxide often the first medicine?
Diazoxide is widely used and specifically indicated for hypoglycemia due to hyperinsulinism. It blocks insulin release and can be taken by mouth. Many children respond well, although some need additional drugs or surgery, and side effects must be watched closely. FDA Access Data+2FDA Access Data+2

5. What happens if diazoxide does not work?
If diazoxide fails or causes serious side effects, doctors often add or switch to somatostatin analogues like octreotide or lanreotide, and may consider mTOR inhibitors or surgery in severe cases. The choice depends on genetic type, age, and hospital experience. Pediatrics Publications+2SpringerLink+2

6. Are somatostatin analogue injections safe for long-term use?
They can be effective but need careful monitoring. Long-term use may cause gallstones, digestive problems, and changes in blood sugar patterns. Regular ultrasound checks, blood tests, and clinic visits help pick up side effects early. FDA Access Data+2FDA Access Data+2

7. Why do some doctors worry about sirolimus or everolimus?
Although these drugs can help in some very severe, resistant cases, they are strong immunosuppressants with many potential short- and long-term side effects. Some experts argue that the risks may outweigh the benefits for many children, so their use is restricted and carefully studied. ScienceOpen+2SpringerLink+2

8. Can diet alone control hyperinsulinism?
For mild or transient forms, careful feeding plans may control blood sugar without long-term medicines. However, many children with genetic congenital hyperinsulinism need both diet and medications, especially in infancy, to stay safe. Congenital Hyperinsulinism International+2Frontiers+2

9. Will my child always need a feeding tube?
Not necessarily. Some children need a gastrostomy tube only during early years when hypoglycemia is hardest to control. As they grow or after successful treatment, they may switch fully to oral feeding and have the tube removed. Others with very severe disease may need longer-term tube support. Children’s Hospital of Philadelphia+1

10. Can my child play sports?
Many children can join sports with planning. Checking blood sugar before, sometimes during, and after activity, providing snacks, and teaching coaches about the condition are important steps. Your team can help create a safe exercise plan. Frontiers+1

11. Is hyperinsulinism always genetic?
Many cases, especially congenital hyperinsulinism in babies, are due to gene changes. However, some forms of hyperinsulinism are secondary to other conditions, drugs, or tumors. Genetic testing and full evaluation help clarify the cause. Frontiers+1

12. Can adults develop hyperinsulinism?
Yes. Adults can develop hyperinsulinism due to insulin-producing tumors (insulinomas), after certain stomach surgeries, or in rare metabolic states. The basic problem is still too much insulin and low blood sugar, but causes and treatment options can be different from those in infants. PMC+2ScienceDirect+2

13. Does hyperinsulinism mean my child will later get diabetes?
Not always, but some children who have a near-total pancreatectomy or long-term pancreatic damage may develop diabetes in later childhood or adulthood because there is not enough pancreas left to make insulin. Long-term follow-up is important to watch for this. Semantic Scholar+1

14. Should I give over-the-counter “sugar control” supplements?
No, not without medical advice. Many such products are designed to lower blood sugar or increase insulin effect, which could be dangerous in hyperinsulinism where low sugar is already the main problem. Always talk to the specialist team first. Medscape+1

15. Where can I find reliable information and support?
Reliable sources include specialist endocrine clinics, published clinical guidelines, and recognized patient organizations for congenital hyperinsulinism. These groups provide updated information, family stories, and practical tools to manage daily life with hyperinsulinism. Frontiers+1

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: December o2 , 2025.