PKU Cooler 10 Neutral the product does not contain the amino acid phenylalanine and is therefore a suitable amino acid, vitamin and mineral source in patients with PKU (phenylketonuria). Foods for special medical nutritional purposes. Are not intended for other than PKU patients.
Active substance
Amino acids. For a full statement see the product datasheet.
Indication
For the nutritional treatment of PKU (phenylketonuria). PKU Cooler is for children 3 years and up. PKU Cooler 15 and 20 are til children from 8 years and adults.
User manual
PKU Cooler can be drunk with the same and should not be mixed with water. It is recommended that PKU Cooler drunk cold. Shake before use. Is PKU Cooler opened the drunk within 24 hours.
Flavours and article
PKU Cooler 10 is also available as:
- PKU Cooler 10 Orange art.nr 90 081
- PKU Cooler 10 forest berries art.nr 90 082
- PKU Cooler Starter Set art.nr 99 978
Packaging
- 1 package Cooler 10 includes 30 x 87 ml.
- 1 package Cooler 15 contains 30 x 130 ml.
- 1 package Cooler 20 contains 30 x 174 ml.
- PKU Cooler (10, 15 and 20) are available as orange, wild berry
About PKU
PKU is a group of recessive hereditary diseases characterized by a deficiency of the hepatic enzyme phenylalanine hydroxylase, which is necessary for the distribution of the essential amino acid phenylalanine into tyrosine. As a result of this deficiency increases plasma phenylalanine concentration and abnormal metabolites are excreted in the urine. This effectively means that the disease affected, the usual protein intake produces high levels of toxins that lead to mental retardation, seizures and defects in pigmentation.
PKU is treated by a diet low in phenylalanine, which should be introduced as soon as possible after diagnosis. Blood phenylalanine is reduced in accordance with MRC Working Party on PKU guidelines. The evidence available so far suggest that children with PKU treated in this manner can develop normally without any symptoms of PKU. It appears, however, that although children with PKU are diagnosed within the first weeks of life and treated with diet according to current guidelines, they have a slightly lower IQ than the norm for the general population.
Intangible earnings are closely related to the quality of diet and blood phenylalanine control during the first decade of life. After adolescence and adulthood is a risk of intellectual deterioration due to high blood pressure. Phenylalanine decreases but it is highly unlikely that this risk no longer exists. Follow-up of early treated children with PKU has shown that diet discontinuation in childhood poses risks of cognitive and emotional disorders in a large number of adolescents and young adults. This dysfunction includes IQ loss, mental processing abnormalities, learning difficulties, anxiety and personality disorders. These changes may be so gradual and subtle that they go unnoticed by the individual for many years or attributed to other causes than high phenylalanine concentration. Family members and close to the patient could be the first to notice changes. It is therefore important that those with PKU who have discontinued the diet did not stop going on PKU clinic.
Only time and careful monitoring of those who have reduced their diet will provide much needed information on the effect of phenylalanine in the adult brain. Until this information is available, there are international agreements that dietary therapy should be continued into adulthood and many centers advocate "diet for life".
PKU pregnancies require specialist management. Pregnancy should be planned and blood phenylalanine concentration controlled in the range 120-250 micromol / L before conception and during pregnancy to protect the fetus from teratogenic effects of phenylalanine. These include mental retardation 92%, microcephaly 73%, congenital heart disease 12%, 40% low birth weight and spontaneous abortion, 24% (Lenke and Levy, 1980). Frequent monitoring and rapid adjustment of the diet along with good communication, support and encouragement are all necessary throughout pregnancy to achieve optimal control and performance.
Follow-up of offspring born to PKU mothers of age 14 years, performing neurological and psychological assessments at timed intervals is recommended.
Phenylalanine is an essential amino acid found in all dietary proteins. In order to reduce phenylalanine intake in people with PKU, it is necessary to replace most of the protein with a mixture of synthetic amino acids with a similar amino acid profile for protein of high biological value, but excluding phenylalanine. Because the diet is very restrictive, PKU diet is also supplemented by a comprehensive range of vitamins, minerals and trace elements.
Traditional sources of protein substitute provides a good source of protein, but they have several disadvantages that may result in poor compliance and poor metabolic control:
- The large volume needed to provide enough protein
- Nuisances in connection with the preparation and transportation out of home
- They are not all nutritionally complete, therefore the patient must take a separate vitamin and mineral supplements
Vitaflo has developed a range of protein substitute for use in PKU, which deals with these drawbacks:
PKU start (Diagnosis to 12 months)
PKU gel (1-10 years) and PKU express (8 years +).
PKU gel and PKU Express is specially designed for low volume nutritionally complete protein substitute that has set new standards for acceptance and comfort in this category of products. It's fast, easy and only takes about a minute. Now available PKU cooling system gives users a new level of convenience.
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