Congenital Hyperinsulinism… Watch your baby!

Congenital Hyperinsulinism… Watch your baby!

Congenital Hyperinsulinism is another reason why you can have high blood insulin levels, and usually occurs in infants or small children. This situation is very dangerous and the worst thing is that it can be easily confused with epilepsy, given that having high insulin levels in the body, causes glucose levels to fall too much, and causing hypoglycemia or low blood sugar.

Hypoglycemia can cause seizures and other symptoms that are characteristic of epilepsy. If hypoglycemia isn’t treated properly or on time, it can lead to many problems, neurological damage or even death. Though there’s much controversy over what the limit value of glycemia is, it’s generally considered appropriate to maintain blood glucose levels above 40 mg/dl on the first 24 hours and above 50 mg/dl on the second day of life, to prevent neurological damage.

It’s vitally important for every baby or child who has a strange behavior, confusion, tremor, sweating or seizures, to be thoroughly examined by an endocrinologist, as these are symptoms of hypoglycemia or low blood glucose levels.

There are multiple causes for congenital Hyperinsulinism. Some are resolved with time and are considered Transient Hyperinsulinism. Others arise from a genetic mutation or alteration in the chromosomes, which are transmitted from parent to child (even if neither parent has the disease) and persist for life, these forms are known as Persistent Hyperinsulinism. Although they won’t disappear, it’s easier to treat them as the child grows. It’s estimated that one in every 25,000 to 50,000 babies are born with Hyperinsulinism and this disease represents the most frequent cause of hypoglycemia during childhood.

A Prenatal Genetic Hyperinsulinism Diagnosis is available nowadays, and is based on analyzing the fetal tissues, which are obtained through amniocentesis. This test is used to obtain a small amount of amniotic fluid from the amniotic sac to then examine the fetal DNA for genetic abnormalities. This allows immediate treatment after birth, aiming to prevent brain damage from hypoglycemia, keeping glucose levels above 70 mg/dl through pharmacological or surgical therapy. This is important because babies who have it, can’t generate alternative substrates (lactate and ketones) to use their brain during a hypoglycemic episode.

These genetic tests are already available through commercial laboratories and are very useful, since it can diagnose approximately 85% of the most severe cases of Hyperinsulinism, and simultaneously, if those cases are identified it’s more likely that they’ll respond positively to drug therapy. Moreover, surgical treatment is prescribed to patients who can’t be controlled with medication but can be cured with surgery.

At Diabetes Up to Date, we are committed to keep you informed about any aspect related to diabetes and its risk factors, so you can get the knowledge you need to enjoy a healthy, productive and happy life.

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Hyperinsulinemia or Hyperinsulinism