03 JUN

How to treat phenylketonuria? How long will it take?

  • Health Fitness
  • Beatrice
  • Mar 18,2022
  • 22

maternal pku

The sooner you maternal pku start treatment, the better. The treatment is mainly "low phenylalanine diet therapy":

Infancy

Feed with low phenylalanine formula first, and then gradually add breast milk or other natural milk after the phenylalanine in the baby's blood decreases. Breast milk is superior to other natural milk, such as milk, because there is less phenylalanine in breast milk than other milk.

If you are breastfeeding, you'd better not use more than 250 ml per day; If it is a milk feeding, the daily intake should not exceed 100 ml. The rest of the time is supplemented by low phenylalanine formula.

Other protein free nutrients should be supplemented as normal children.

After 1 year old

After one year of age, you still want to give your child enough low phenylalanine formula, but don't drink milk and other natural milk.

At this time, children can eat a variety of foods. They should pay attention to choosing low protein foods, such as vegetables, fruits, potatoes, lotus root powder, noodles, noodles, steamed bread, sugar, etc. The protein content of rice is a little high. Don't exceed one or two a day.

puberty

The treatment of low phenylalanine diet should last at least until adolescence (about 12 years old). After adolescence, diet control can be relaxed appropriately. Of course, if you can stick to diet control all your life, it will be better for your health.

In addition to "low phenylalanine is treated by diet", tetrahydrobiopterin (BH4) supplementation may be due to mild hyperphenylalaninemia (blood phenylalanine ion concentration is 150-600 μ Mol / L) can replace the traditional treatment research method.