Younger infants: low-phenylalanine milk as a base, which pku guidelines can be added to breast milk or supplementation as appropriate
Breastfeeding should be stopped after diagnosis, but weaning should not be done to control blood phenylalanine levels in a timely manner.
Children are treated with low-phenylalanine formula at doses based on protein requirements per kg body weight
During the 4 weeks of treatment, blood samples were taken once a day within 2 hours of taking this special milk formula and blood phenylalanine concentrations were measured.
About 4 days after treatment, the blood phenylalanine dropped below 600 μmolu002Fl. When the blood phenylalanine concentration drops to the ideal level, a small amount of natural diet can be added gradually, with breast milk being the first choice.
Around 6 months of age, you can add appropriate rice soup, egg yolk, etc. The addition of egg yolk starts from 1/4, one egg yolk is about 18-20g, containing about 100-120mg of phenylalanine.
Preventive measures
(1) through prenatal examination and diagnosis, newborn screening, reduce the number of births or early diagnosis and treatment of affected families.
Newborn screening has been gradually promoted and popularized in our country, and screened children can often be diagnosed and treated within 4 months or even 2 weeks after birth, which provides protection for the healthy growth of children.
(2) During premarital medical checkups, PKU carriers are screened for family members with PKU, which reduces the birth rate of children with PKU.
(3) For female patients who are planning to get pregnant, strict dietary treatment should be started in the first half of pregnancy. This is until the fetus is born.
Otherwise, high levels of phenylalanine in the blood can cause congenital damage to the fetus.
Although quick treatment beginning during prenatal metabolic screening is crucial for good development and the avoidance of disability, the significant cognitive damage associated with untreated PKU can sometimes be partially overcome with dietary treatment.
Babies with PKU must instead consume newborn formula free of phenylalanine because both breast milk and conventional infant formula do. The amount of breast milk or ordinary formula to be added to the phenylalanine-free formula can be precisely calculated by a dietician.
It is acceptable to utilize foods like yoghurt, cow's milk, morning cereals, rice, peas, spinach, broccoli, and potatoes to make up the daily protein allowance for protein tolerance up to 10 g (500 mg) (phenylalanine).
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