PKU Diet - Phenylketonuria

Phe metabolic diet the primary medical therapy for people living with PKU.

This App to families and children with PKU on phenylalanine restricted diets • PKU Diet manager is the Phe | Protein | Kcal | Carbonates | Fats | Protein Equivalent Calculator.

Because food labels do not indicate the phenylalanine content of foods compared to protein content. It is very difficult to track its daily consumption. In the app, you can:
○ see some basic products;
○ manager the basic composition of food: kilocalories, protein, phenylalanine substitute (equivalent) of protein, carbonates & fats;
○ add a certain amount of Phe separately from the food;
○ create your own product;
○ edit and change parameters;
○ сopy days along with food weights;
○ editing the product included in the diet;
○ template for the number of meals;
○ view the main composition of the product per 100 grams and per entered grams;
○ calculation of product indicators when added to the diet: you can now enter grams • phenylalanine • protein. All fields are recalculated automatically;
○ monitoring of amino acid levels and composition for each product individually, meals, and for the whole day.

Daily Phe counting and meal planning:
Treatment is with a diet low in foods that contain phenylalanine and special supplements. The diet should begin as soon as possible after birth and be continued for life.

Calculation of Phenylalanine vs Protein:
Nutrition labels on foods do not list Phe content of foods, it is very difficult for peoples to track daily dietary Phe intake.

Food base and add personal lists:
People who are diagnosed early and maintain a strict diet can have normal health and a normal life span.

○ Info.....
Phenylketonuria (PKU) is an inborn error of metabolism that results in decreased metabolism of the amino acid phenylalanine. Untreated, PKU can lead to intellectual disability, seizures, behavioral problems, and mental disorders. It may also result in a musty smell and lighter skin. A baby born to a mother who has poorly treated PKU may have heart problems, a small head, and low birth weight.

Phenylketonuria is a genetic disorder inherited from a person's parents. It is due to mutations in the PAH gene, which results in low levels of the enzyme phenylalanine hydroxylase. This results in the buildup of dietary phenylalanine to potentially toxic levels. It is autosomal recessive, meaning that both copies of the gene must be mutated for the condition to develop. There are two main types, classic PKU and variant PKU, depending on whether any enzyme function remains. Those with one copy of a mutated gene typically do not have symptoms. Many countries have newborn screening programs for the disease.

Treatment is with a diet low in foods that contain phenylalanine and special supplements. Babies should use a special formula with a small amount of breast milk. The diet should begin as soon as possible after birth and be continued for life. People who are diagnosed early and maintain a strict diet can have normal health and a normal life span. Effectiveness is monitored through periodic blood tests. The medication sapropterin dihydrochloride may be useful in some.

Males and females are affected equally. The disease was discovered in 1934 by Ivar Asbjørn Følling, with the importance of diet determined in 1953.


♥ Create a perfectly balanced menu