Understanding the Deficiency of Biotinidase
Once again, the Mother Who Loves Portal brings to the mother a disease that can reach her children from birth, the Deficiency of biotinidase .
Biotin is a necessary vitamin for the proper functioning of our body. The complexion of the human body is remarkable because many of the substance provided to our organism can come from food, the air we breathe, and even sunlight. Biotin fits as one of these elements.
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Biotin is regulated by the biotinidase enzyme, which is responsible for the vitamin cycle separating biotin from food proteins. In this way, the enzyme also allows biotin to be recycled several times, reducing the need for daily consumption.
This vitamin is particularly essential because it allows the carboxylase enzymes to perform their function, influencing the metabolism of some types of carbohydrates, fats and proteins.
Therefore, we have two co-related enzymes, one is the biotinidase responsible for regulating biotin. The other is the carboxylase that requires this biotin for the proper functioning of the metabolism of carbohydrates, fats and proteins.
When there is a deficiency in one of the enzymes, in this case, the biotinidase, breaking all this cycle due to the malfunction of biotinidase. Thus, the biotin in food can not be used by the carboxylases. This congenital problem leads to the need for a diet with a lot of free biotin in the body since in its absence, problems begin to appear in the body in the most varied degrees, so stay tuned!
Symptoms of biotinidase deficiency
The lack of biotin in a child can have several consequences on his health and development. As a result, the child may present growth retardation, skin rashes, hearing loss, speech problems, and others.
As the disease can present singular symptoms, it is difficult to clinical diagnosis, when the examination is after the birth of the child because the disease can be diagnosed as early as the first days of life by the test of the foot.
The test is a right of every citizen and should be applied to every child in the territory of the Brazilian federation, read more about it here . The following is a list of other possible manifestations of the disease that denounce biotin deficiency.
- Severe hair loss (from the root);
- Hypotonia;
- Accumulation of lactic acid in the body;
- Loss of muscle coordination;
- Conjunctivitis;
- Vision problems;
- Tiredness;
- Excess of ammonia in the blood;
- Breathing problems;
- Fungal infections;
- Difficulty in feeding;
- Liver enlargement;
- Spleen enlargement;
These symptoms do not have a certain date to appear. There are cases of previous manifestations and others very late, but usually present during the third and sixth month of life.
It is noteworthy the severity of this congenital disease: the deficiency of undiagnosed and treated biotinidase can be classified as one of the causes of Sudden Death Syndrome in Childhood, but still need further studies for this fact.
How is the treatment?
The treatment itself is extremely simple. However, the increased risk and side effects are due to lack of diagnosis and treatment of enzyme deficiency in biotinidase. Therefore, it can be supplied with the direct intake of biotin. In children who were diagnosed early by the foot test , the incidence of health damage is practically nil. It is characterized by being the disease triada in the neonatal easier, cheaper and effective to take care of.
So stay tuned, it's always good to remember that just doing the little test can change a whole life!