Friday, May 19, 2017

Biotinidase Deficiency: Early Diagnosis

This inherited disorder causes the body to be unable to recycle the vitamin biotin.

Today’s article will be a part of a series of topics related to disorders and affections of children in their early stages of growth. Most of these are well known, like stuttering and other disorders that are not so common.
Biotinidase deficiency disorder is an inherited disease that requires treatment and constant care from parents.

Biotinidase is an enzyme that allows the body to recycle the B vitamin biotin. Biotinidase is in charge of extracting the biotin from the food in its free form. This enzyme is also responsible for recycling the biotin from enzymes in the body that use it to function properly. Biotin, also known as vitamin H, is an essential vitamin that helps in the metabolism of proteins, carbohydrates, and proteins.

This deficiency occurs in one out of every 60,000 births. The condition is more common among individuals of European descent, but it also has been reported among Japanese, Saudi Arabian and Turkish, as well.

The problem of biotinidase deficiency (BIOT) is that it disables the reuse and recycling of the vitamin H in the body. There are two types of BIOT: severe “profound biotinidase deficiency” and the partial, milder form of the condition.

The lack of biotin in newborns starts to show months after their birth; these are the early signs of BIOT:
  • Weak muscular tone
  • Skin Rash
  • Hair loss
  • Candidiasis: a fungal infection
  • Seizures

Biotinidase deficiency doesn’t have a cure, but it can be treated. Children with BIOT often require a lifelong treatment of biotin supplements to compensate the lack of it in their bodies. The dosage of biotin will be determined by the doctor and will vary depending on the type of BIOT.

The biotinidase deficiency needs to be spotted and treated early to avoid complications in the children’s growth. If the treatment is constant, your kids will develop normally and be able to live like any other healthy children.
As they have more difficulties in getting their nutrients, they need a lifelong treatment with biotin supplements.


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