Question

Phenylketonuria (PKU) is a recessive human disorder in which an individual cannot appropriately metabolize the amino acid phenylalanine. This amino acid is not naturally produced by humans. Therefore, the most efficient and effective treatment is which of the following?a.Transfuse the patients with blood from unaffected donors.b.Regulate the diet of the affected persons to severely limit the uptake of the amino acid.c.Feed them the substrate that can be metabolized into this amino acid.d.Feed the patients an excess of the missing product.e.Feed the patients the missing enzymes in a regular cycle, such as twice per week.

Answer 1

b. Regulate the diet of the affected persons to severely limit the uptake of the amino acid.

Step-by-step explanation:

In almost all countries, newborn babies are routinely tested for phenylketonuria, since it is important to apply a special diet for life. It is an uncurable disease, but the effects can be regulated controlling the phenylalanine ingest, therefore its accumulation in the organism. Some of the products that needs to be avoided are meat (chicken, pork, beef, fish), milk, eggs, dairy products, nuts, soybeans, beans. This means that also for life the patients need to get a nutritional supplement to ensure that they get enough essential protein, without the phenylalanine.