2. Clinical test of Phe: The monitoring of blood Phe concentration after diet therapy should be reviewed once or twice a week in the first month, and once a month thereafter.
3, protein, calories should be enough: how to control the blood phenylalanine concentration of children with PKU, and whether the supply of protein and calories is sufficient is the key to diet therapy. If protein and heat supply are insufficient, it will cause nutritional deficiency, decompose protein in the body, and also increase blood Phe.
4. Importance of breast milk: Breast milk is still the best diet for children with PKU. Giving a calculated amount of breast milk is very beneficial to children's development. Never stop breastfeeding.
5. Milk concentration adjustment: The standard milk concentration adjustment is generally 15%, but its osmotic pressure is higher than that of body fluid. The milk concentration adjustment of milk powder in lactation period, especially for newborns, should not be too high. At the same time, it is emphasized that water should be fed between milkings to replenish water.
6. Taste problem: Most PKU children are intolerant of the unique taste of amino acids in therapeutic milk powder. Consider adding therapeutic milk powder to orange juice, orange juice, apple juice and other juices for drinking.
7. Milk powder and protein powder: phenylalanine-free milk powder basically contains all kinds of nutrients needed by infants, but the protein content is low, which is mainly used for PKU children within 1 year old; However, the protein content of phenylalanine-free protein powder is high, but other nutrients should be supplemented by other diets, which are mainly used for PKU children over 1 year old.
8. Food diversification: development depends on good eating, and high-quality protein (eggs, meat and fish) is selected as the source of phenylalanine. Appetite depends on the diversification of diet (steamed bread, rice, steamed buns, jiaozi, oil cakes, noodles, pilaf, cakes ... all should be specially made).
9. Vitamins and trace elements: Fully supply fruits and vegetables to meet the needs of vitamins and trace elements, and can correct constipation.
1. Carnitine: The synthesis ability of carnitine in PKU children is low. Exogenous carnitine mainly comes from meat and dairy products, and PKU children need to add it to their diet because of limited sources.
11. classification of PKU: the increase of blood Phe concentration at the initial diagnosis can infer the degree of Phenylalanine hydroxylase deficiency in the liver, which probably suggests the classification of PKU and the appropriate intake of phe.
12. Food Composition List: Bring your own Food Composition List. When the blood concentration is normal, choose the food with phenylalanine content below 1 in Table 2. When the blood concentration is high, choose the food with phenylalanine content below 5 to feed; When the blood concentration is low, eggs, meat foam, yogurt, ice cream and other foods can be added from less to more.
13. Aspartame: All beverages, sweets and cakes containing artificial sweeteners containing phenylalanine such as aspartame are inedible. Honey contains phenylalanine 44mg/1g, which can be selected according to the calculated amount.
14. Psychological and educational problems: Pay attention to the changes of psychology and behavior, find out psychological diseases in time, and go to the psychiatric department for medical treatment in due course. After the child has eaten enough, others will eat again. Can make children develop the self-discipline habit of special diet. In order to prevent children from stealing or receiving normal food from others. Relatives and friends must accept PKU's living conditions, overcome the ordinary thinking of "poor children", and can't instigate children to taste ordinary food.
15. Complications: Children with other complications should be treated symptomatically. For example, children with epilepsy should start regular antiepileptic drug treatment as soon as possible. Children with eczema can heal themselves after the blood Phe concentration is controlled satisfactorily. If eczema is serious, they can be treated with topical medicine.
16. Atypical phenylketonuria: In addition to diet therapy, the treatment of atypical phenylketonuria should also be supplemented with a variety of nerve mediators, such as BH4, dopa, 5- hydroxytryptamine and folic acid.
17. Rehabilitation training: The mental retardation of people with brain injury is irreversible, but if they undergo mental rehabilitation, they will also improve in different degrees, and some may even make significant progress. For families where conditions permit, mental rehabilitation training can be considered for children. For children with severe mental retardation, the purpose of training is to cultivate their basic self-care ability. For children with mild and moderate mental retardation, on the basis of cultivating their living ability, they should also be trained in corresponding survival skills.