Hyperinsulinism represents a condition of increased insulin concentration in the blood, which results in hypoglycemia (low blood sugar). Hypoglycemia often causes the most severe health problems, which can lead to coma or even death.
What is hyperinsulinism?
There is a distinction between hyperinsulinism and hyperinsulinemia. Whereas in hyperinsulinemia, insulin concentrations are only temporarily elevated, hyperinsulinism is characterized by permanently elevated insulin levels in the blood. There are several causes for the elevated concentrations in the blood. The most common is congenital hyperinsulinism, which is genetically determined and already exists from birth. Insulin, as a hormone of the pancreas, is responsible for regulating blood glucose levels. It ensures the introduction of glucose into the body’s cells. The more insulin is present, the more glucose is transported into the cells. The blood glucose level drops more and more and reaches values that are too low. Therefore, the body can no longer be adequately supplied with glucose. The brain in particular is dependent on glucose. If the glucose supply to the brain becomes too low, it can no longer perform important functions adequately. In severe cases, coma and death often occur. In milder cases of hyperinsulinism, the body tries to compensate for hypoglycemia by increasing food intake.
Causes
Hyperinsulinism can be caused either by permanently increased insulin production in the pancreas or by delayed breakdown of insulin. In most cases, however, insulin production is increased. For example, type II diabetes is caused by decreased insulin resistance. This means that although there is enough insulin, it is less effective due to a lack of insulin receptors. The pancreas now tries to compensate for insulin effectiveness by increasing insulin production. In this case, however, blood glucose levels remain elevated or reach maximum normal levels. Hyperinsulinism with hypoglycemia develops in the case of a specific tumor of the pancreas (insulinoma) or in the case of a genetically determined overproduction of insulin. So-called genetic congenital hyperinsulinism is the most common form of hyperinsulinism. This in turn can be divided into focal and diffuse hyperinsulinism. In focal congenital hyperinsulinism, there is usually only one affected site in the pancreas. Diffuse hyperinsulinism is characterized by increased insulin production by all islet cells. Insulin production may also be increased by psychological influences or by a pancreas that is easily reactive.
Symptoms, complaints, and signs
Hyperinsulinism is characterized by constant sweating, tremors, as well as pallor. In congenital hyperinsulinism, these symptoms appear from birth. Furthermore, behavioral abnormalities, lethargy, seizures, and impaired consciousness are observed. The symptoms can be treated quickly by glucose administration. Afterwards, however, the blood glucose level drops again very sharply. In severe untreated cases, the condition can be fatal or cause severe mental developmental disorders. In the very mild forms of hyperinsulinism, a constant feeling of hunger often leads to increased food intake. As a result, high-grade obesity may occur.
Diagnosis and course of the disease
Hyperinsulinism is diagnosed by blood tests. Insulin levels, fasting blood glucose in the blood, and ketone bodies in the serum are measured. If fasting insulin levels are above 3 U/l and blood glucose levels are decreased, hyperinsulinism can be said to exist. The concentration of ketone bodies is lowered because insulin inhibits lipolysis and thus fewer ketone bodies can be produced from the breakdown of fatty acids. Improvement of symptoms by glucose administration or administration of glucagon also indicates hyperinsulinism. Finally, imaging techniques such as sonography can be used to assess pancreatic tissue. In addition, sonography is also useful in ruling out a tumor as a cause of hyperinsulinism. Assessment of the pancreatic parenchyma involves distinguishing focal from diffuse hyperinsulinism. The distinction is very important for designing the treatment strategy.
Complications
Hyperinsulinism causes the patient to become severely hypoglycemic. This can lead to various health complaints and complications. In many cases, people lose consciousness or feel ill and fatigued during hypoglycemia. The patient’s ability to cope with stress decreases considerably and the heartbeat increases even during light activities. The affected person also appears pale and can hardly concentrate. Disturbances of consciousness occur and the patient suffers from sweating and seizures. The quality of life is considerably reduced by hyperinsulinism. If the disease occurs in children, it can lead to significant disturbances in development, which can usually lead to consequential damage in adulthood. The feeling of hunger is intensified by hyperinsulinism and the patient tends to increase food intake, which can lead to the development of obesity and overweight. There are no particular complications in the treatment of the disease. Mainly medications are used, which can eliminate the symptoms relatively quickly. Only in rare cases surgical intervention is necessary. With early treatment, the life expectancy of the affected person is not reduced.
When should you go to the doctor?
People who basically sweat rapidly even when making slight movements or performing everyday tasks should see a doctor for clarification of the symptoms. Nervousness, persistent trembling of the limbs or a pale facial coloration are indications of discrepancies. A visit to the doctor is necessary as soon as the symptoms persist over several days or weeks and increase in intensity. Cramping of the body, abnormal behavior or lethargy should be examined by a doctor. If the symptoms already occur in a newborn child, this must be reported to the pediatrician immediately. In case of disturbances of consciousness or unconsciousness, medical care must be given as soon as possible to ensure the survival of the affected person. In severe cases, an emergency physician must be called. Until his arrival, first aid measures must be taken and the breathing supply must be secured. If there is a sudden collapse, unexpected behavioral abnormalities or severe mood swings, there is cause for concern. If there is a drop in the usual level of performance, reduced drive and inner restlessness, a visit to the doctor is advisable. General weakness, a strong feeling of hunger and disturbances in food intake should be discussed with a doctor. If there are irregularities in digestion, changes in weight, or an increased need for sleep, the observations should be investigated by a physician.
Treatment and therapy
Treatment of hyperinsulinism depends on its cause. Conservative treatment methods include oral or intravenous glucose substitution. In addition, the hormone glucagon can also be substituted. Glucagon is the antagonist of insulin and ensures the breakdown of glucogen stored in the liver into glucose. Drug treatment with diazoxide or nifedipine can be given at the same time. Diazoxide opens the ATP-sensitive potassium channels. Nifedipine provides inhibition of calcium channels. This inhibits insulin secretion. If conservative methods fail to improve symptoms, surgical removal of the overactive islet cells may also be considered. In the case of focal hyperinsulinism, a complete cure is thus often possible. Here, only the affected area of the pancreas is removed. In diffuse hyperinsulinism, complete resection of the islet cells would have to be performed. In this case, however, type I diabetes develops. Therefore, in this case, an attempt is made to achieve the greatest possible success through drug treatment so that surgery is not necessary. However, it has been found that there is still an atypical hyperinsulinism, which combines both forms of congenital hyperinsulinism. In atypical hyperinsulinism, there are multiple highly active sites in the pancreas. Imaging techniques can detect these. Again, surgical removal of the affected sites can bring about a complete cure, if necessary.
Outlook and prognosis
Hyperinsulinism triggers serious complications if medical care is not sought.The prognosis in these cases is extremely unfavorable. Hypoglycemia and severe life-threatening consequences occur. The patient may lose consciousness and fall into a coma. This condition is potentially life-threatening or may result in irreparable damage. With treatment, the prospects of alleviating the symptoms improve. Nevertheless, the cause of the hyperinsulinism is decisive. In the case of tumor disease, the stage and curability of the tumor are decisive for the overall prognosis. If the tumor is discovered early and completely removed, a good prognosis can be given with subsequent cancer therapy. In most cases, long-term therapy is subsequently initiated, with a significant improvement in symptoms. If there is no prospect of a cure for the cancer therapy, drug treatment takes place to alleviate existing complaints. In these cases, the patient’s care is focused on symptom relief of all irregularities to alleviate existing suffering. If the patient suffers from an underlying chronic disease, no cure is expected. Insulin balance is monitored and regulated. As soon as prescribed drugs are discontinued, the symptoms relapse. If the treatment plan is followed, there is a sustained improvement in quality of life.
Prevention
Prevention is not possible for most forms of hyperinsulinism. Only hyperinsulinism caused by type II diabetes mellitus can be prevented by a healthy lifestyle.
Follow-up
In most cases of hyperinsulinism, follow-up proves to be difficult. In this regard, the measures and the possibilities of aftercare are severely limited in most cases, so that the affected person is primarily dependent on a quick and, above all, an early diagnosis of this disease. In the worst case, untreated hyperinsulinism can even lead to the death of the affected person, so that early detection is the most important factor in this disease. The earlier hyperinsulinism is detected, the better the further course of this disease usually is. Often, the disease is treated by taking medication. It is important to ensure that the correct dosage is taken regularly in order to alleviate the symptoms properly and permanently. If there are any questions or uncertainties, a doctor must always be consulted first. A doctor must also be consulted first in the event of side effects or drug interactions. Contact with other patients of hyperinsulinism can be useful, as it is not uncommon for information to be exchanged. Whether there will be a decreased life expectancy due to the disease cannot be predicted in general.
This is what you can do yourself
Hyperinsulinism definitely needs to be treated by a doctor. In the worst case, without treatment, the patient may die. In case of diabetes disease, a healthy diet and lifestyle can have a very positive effect on the disease. Ideally, this can also limit the disease completely. As a rule, however, those affected are dependent on medical treatment. Since sufferers sweat frequently and heavily due to the disease, light and airy clothing should be worn to avoid sweating. In the case of behavioral abnormalities or disorders of consciousness, special support for the affected person can have a positive effect on the disease. However, the symptoms can be treated relatively easily and quickly by taking medication. Furthermore, patients with hyperinsulinism should participate in regular blood tests to control the blood glucose level. Other examinations of the internal organs are also advisable. In case of loss of consciousness during hypoglycemia, an emergency physician should be called. Until the emergency physician arrives, the affected person should be placed in a stable lateral position. Regular and calm breathing should also be maintained.
