Why Does Aspartame Have a Warning Label?

Discussion Phenylketonuria (PKU) is an autosomal recessive genetic disease. The PAH gene is found on chromosome 12 and has more than 600 mutations associated with it. The incidence varies but PKU is primarily found in Caucasian populations. The PAH gene codes for phenylalanine hydroxylase (PAH) which catalyzes the amino acid phenylalanine (Phe) to tyrosine. PAH uses a cofactor called tetrahydrobiopterin (BH4) in this process. With a deficiency in PAH, Phe accumulates and can cause severe cognitive impairment and global developmental delay, microcephaly, seizures, poor growth and poor skin pigmentation. Tyrosine also decreases and along with it its products of melanin, L-thyroxine and catecholamine neurotransmitters. The exact cause of the PKU clinical problems may be an accumulation of Phe, deficiency in Tyrosine products or another cause but is unknown exactly currently. The mainstay of treatment is a low protein, low phenylalanine diet. Phe is an essential amino acid so some Phe must be supplied in the diet but the amount is highly individualized based on the specific mutation, age and other specific needs of the individual. Specialized formula that is Phe-free is used for infants with children and adults using similar alternative formulas as part of their overall diet. The diet centers on consuming mainly fruits and vegetables. Low protein grains are also used, along with much smaller amounts of whole proteins along with Phe-free formulas. Food products for medical d...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news