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Pheylketonuria (PKU) is a genetic disorder in which the body is not able to process an amino acid called phenylalanine, according to MedlinePlus.com 1. High levels of phenylalanine can damage the brain and lead to mental retardation. All babies born in the U.S. must have a screening test for PKU. Phenylalanine is mostly found in high-protein foods. High-protein foods should be avoided by patients with PKU.
Fish, Poultry, Red Meat and Dairy Products
Brain damage can be prevented if patients with PKU are treated with a special diet that is low in phenylalanine. In the PKU diet, animal protein is avoided because it contains the highest amounts of phenylalanine. Examples of animal protein to be avoided include red meat, fish, poultry and milk and milk products. PKU patients are to eat low-protein foods such as low-protein breads, pastas and cereals. Babies with PKU are often fed a special formula containing high protein and low phenylalanine. People with PKU who are on this special diet from birth or shortly thereafter develop normally and often have no symptoms of PKU, according to MedlinePlus.com 1.
- Brain damage can be prevented if patients with PKU are treated with a special diet that is low in phenylalanine.
- In the PKU diet, animal protein is avoided because it contains the highest amounts of phenylalanine.
Dried Beans and Peas and Nuts
Patients with PKU should avoid vegetable protein such as dried beans, peas and nuts as they contain high amounts of phenylalanine, according to MedlinePlus 1. Examples of nuts to avoid include:
PKU patients are usually advised to eat a lot of fruits and vegetables because they contain little phenylalanine.
Patients with PKU should avoid products containing the artificial sweetener aspartame, according to MedlinePlus 1. When digested, aspartame produces amounts of phenylalanine considered dangerous to PKU patients. PKU patients should make sure they read food labels before buying these products.
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- MedlinePlus: Phenylketonuria
- University of Washington: PKU Diet
- Marble, M. Phenylketonuria and other metabolic diseases: clinical, genetic and newborn screening aspects. LSU Health Sciences Center.
- Burlina AP, Lachmann RH, Manara R, et al. The neurological and psychological phenotype of adult patients with early-treated phenylketonuria: A systematic review. J Inherit Metab Dis. 2019;42(2):209-219. doi:10.1002/jimd.12065
- Hood A, Antenor-dorsey, JA, Rutlin J, et al. Prolonged exposure to high and variable phenylalanine levels over the lifetime predicts brain white matter integrity in children with phenylketonuria. Mol Genet Metab. 2015;114(1):19-24. doi:10.1016/j.ymgme.2014.11.007
- Macleod EL, Ney DM. Nutritional management of phenylketonuria. Ann Nestle Eng. 2010;68(2):58-69. doi:10.1159/000312813
- University of Washington, Cristine M. Trahms Program for Phenylketonuria. What is the diet for PKU?
- Sara, GL., Alejandra, LML, Isabel, IG, Marcela, VA. Conventional phenylketonuria treatment. Journal of Inborn Errors of Metabolism and Screening. 2010;4. doi:10.1177/2326409816685733
- Southeast Regional Genetics Network. PKU nutrition management guidelines. Updated August 2016.
- Kose E, Aksoy B, Kuyum P, Tuncer N, Arslan N, Ozturk Y. The effects of breastfeeding in infants with ohenylketonuria. J Pediatr Nurs. 2018;38:27-32. doi:10.1016/j.pedn.2017.10.009
- Guo H, Zheng Y, Wang B, Li Z. A note on an improved self-healing group key distribution scheme. Sensors (Basel). 2015;15(10):25033-8. doi:10.3390/ijns5030026
- Concolino D, Mascaro I, Moricca MT, et al. Long-term treatment of phenylketonuria with a new medical food containing large neutral amino acids [published correction appears in Eur J Clin Nutr. 2017 Aug;71(8):1027]. Eur J Clin Nutr. 2017;71(1):51–55. doi:10.1038/ejcn.2016.166
- Kose E, Aksoy B, Kuyum P, Tuncer N, Arslan N, Ozturk Y. The effects of breastfeeding in infants with phenylketonuria. J Pediatr Nurs. 2018;38:27-32. doi:10.1016/j.pedn.2017.10.009
- Southeastern Genetics Network. PKU nutrition management guidelines.
- The National PKU Alliance. Returning to diet.
- University of Washington. Cristine M. Trahms Program for Phenylketonuria. What is the diet for PKU?
Esther Kinuthia is a registered nurse with extensive experience in health and wellness. She holds a B.S. in nursing, B.A in psychology and has worked for more than ten years in the health-care field. She enjoys writing articles on a variety of topics for the Internet. Her work has been published in various websites.