CONGENITAL HYPERINSULINISM

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Congenital hyperinsulinism (HI) is a rare disease that causes those affected to be permanently at risk of having “blood sugar drops”.

In HI, insulin is “blind” to the level of blood glucose and the body continues to release it even if it has not enough energy to function properly.

In addition, in HI, insulin also prevents other alternative energy sources from being used.

This is especially dangerous, because low blood sugar
can cause irreversible neurological damage,
coma and even death.

HOW IS IT SUPPOSED TO BE?

Our body needs energy to function properly. Yet, our brain is the part of our body that uses the most energy. Normally, this energy is provided by glucose (commonly known as “sugar”).

Insulin is a hormone that is produced in the pancreas and helps the body in controlling the available energy.

Insulin is the key that allows glucose to enter into cells. The main objective is to store energy reserves so they can be used later.

This energy is stored in the liver, muscles and fatty tissue.

Under normal conditions, blood sugar levels stay between 70-150mg / dl.

Although health professionals do not completely agree with the values of hypoglycemia, it is generally considered that values below 70 mg/dl are not recommended.

During digestion, carbohydrate-containing foods are converted into sugars and passed from the small intestine to the blood vessels.

When the concentration of glucose in the blood increases, the beta cells of the pancreas secrete insulin and pour it into the blood vessels.

In a healthy person, insulin secretion response is immediate when blood sugar levels rise.

When blood sugar levels drop, the body activates defense mechanisms against hypoglycemia. Firstly, glucagon is released; it is the hormone that recovers glucose stored in the liver to return it to the blood.

If everything works well, our body can also counteract hypoglycemia by recovering glucose stored in muscles and fat.

AND WHAT HAPPENS IN HI?

HI is a set of alterations that affect the mechanism of insulin secretion.

Although there are many different causes, all those affected by HI have frequent episodes of hypoglycemia.

In HI, insulin, in addition to reducing blood glucose levels, also prevents the activation of mechanisms to use alternative energy sources. This means that the brain does not have any source of energy when blood sugar levels fall. Obviously, this is unsustainable because in the same way that a car can not function without fuel, our brain does not work properly if the glucose that reaches it is insufficient. When this is the case, short-term and long-term effects may occur.

Babies and children exposed to continuous hypoglycemia have been shown to suffer changes in their brain connections, which can cause irreversible neurological damage. Brain damage caused by low blood sugar can make children have learning difficulties or have seizures.

But there is still more: if hypoglycemia is not corrected in a timely manner, it can endanger even the person’s life.

Therefore, a rapid and timely diagnosis and adequate treatment to avoid severe hypoglycemia are essential in HI.

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SOME RELEVANT DATA ABOUT CONGENITAL HYPERINSULINISM

%
OF BABIES SUFFER FROM HYPOGLYCEMIA
WITHIN THEIR FIRST MONTH OF LIFE
%
OF PEOPLE AFFECTED BY HI HAVE LOW BLOOD
SUGAR WITHIN THEIR FIRST YEAR OF LIFE
%
OF PEOPLE AFFECTED BY HI HAVE NEUROLOGICAL DAMAGE

If you want to learn more…

If you want to expand the information on congenital hyperinsulinism we recommend you visit the following websites:

La Otra Cara de la Insulina reminds you that we are not doctors. 
If you have any doubts or questions about treatment, 
you should consult qualified medical personnel.

No one wants to come across an illness in their life, much less if it is a disease of their child.

However, sometimes storms give way to sunny days.

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