PKU is a rare metabolic disorder that disrupts the metabolism of the amino acid phenylalanine. If not treated, the level of phenylalanine builds up in the blood and poisons nerve cells (neurons) in the brain, causing mental retardation.
There is no cure for PKU, but treatment can prevent intellectual disabilities and other health problems. People with PKU need to follow a special dietary regime that limits foods with phenylalanine. The diet should be followed carefully and be started as soon after birth as possible.
The current PKU diet consists of a low protein diet and free amino acids supplements in order to limit the patients intake of phenylalanine.
Improved nutritional management of PKU with Lacprodan® CGMP-20
Lacprodan® CGMP-20 is a highly purified casein glycomacropeptide product that is very suitable as a protein source for PKU patients since it is very low in the amino acid phenylalanine. Lacprodan® CGMP-20 can substitute the main part of the free amino acids in the PKU diet and provides several beneficial nutritional effects compared to free amino acids.
The fact that Lacprodan® CGMP-20 is a peptide ensures that the absorption rate of its amino acids is prolonged compared to free amino acids and thereby results in improved protein retention and increased satiety compared to free amino acids. Another important benefit of Lacprodan® CGMP-20 is that the taste is significantly improved when it substitutes part of the free amino acids and this may help ensure high patient compliance to the PKU diet.
Lacprodan® CGMP-20 furthermore contains a very high level of large neutral amino acids, such as threonine and isoleucine, which compete for uptake into the brain with phenylalanine and will help maintain plasma phenylalanine levels in the desired target range.