Phenylalanine can cause intellectual disabilities, brain damage, seizures and other problems in people with PKU. A phenylalanine screening test identifies levels of phenylalanine in your blood. Simply put, "Phenylketonurics" means "People who suffer from Phenylketonuria", whilst "phenylalanine" is the name of a particular essential amino acid which cannot be made in the human body. 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Depending on the severity of the PKU, babies who were not treated for the disorder and put on the special diet at birth run the risk of suffering irreversible mental retardation within the first year of life. Phenylketonuria. Phenylketonuria. time. If you or your child develops symptoms of phenylketonuria, contact your healthcare provider for a thorough evaluation. For the rest of their lives, people with PKU babies, children and adults need to follow a diet that limits phenylalanine, which is found mostly in foods that contain protein. The adverse reactions that seem to be caused by aspertame sensitivity include headaches, muscle pains, skin rashes, hypertension, lethargy and heart palpitations. It is believed that aspartame can cause a wide range of side effects - from stomach problems, to headaches, nausea and skin rashes. information and will only use or disclose that information as set forth in our notice of These amino acids are then used to make our own proteins. Good sources include high protein foods. at newsletters@mayoclinic.com. Humans cannot make phenyalanine, but it is a natural part of the foods we eat. Many rare diseases have limited information. If you don't have PKU, you probably don't need to worry about harmful health effects of phenylalanine with certain important exceptions. other information we have about you. Instead, it comes from the digestion of one type of artificial sweetener, called aspartame, that is used in some chewing gums for flavoring. So, what is PKU? This is achieved through diet formulations, protein supplements, and enzyme substitutions. But what is phenylketonurics? People with PKU have a deficiency of an enzyme which is necessary for the proper metabolism of an amino acid called Phenylalanine (i.e., the component in diet soda included in the warning). .st3 { Accessed July 3, 2018. Sign up for free, and stay up to date on research advancements, health tips and current health topics, like COVID-19, plus expertise on managing health. Some medications may contain aspartame and some vitamins or other supplements may contain amino acids or skim milk powder. Thats why babies in America are usually tested for PKU when they are born. Phenylalanine - Aspartame. Cleveland Clinic Community Care puts patients first by offering comprehensive, coordinated, personalized healthcare. Aspartame is a widely used artificial sweetener and a popular sugar substitute in low-calorie food and drinks, including diet sodas. The gene affected, PAH, encodes phenylalanine hydroxylase, which converts phenylalanine to tyrosine (another amino acid) in the liver. If women don't follow the special PKU diet before and during pregnancy, blood phenylalanine levels can become high and harm the developing baby. This gene helps create the enzyme needed to break down phenylalanine. If you have PKU and you eat any protein food or a product with aspartame, it would cause serious health issues. Some people seem to be particularly sensitive to aspartame, though the incidence and range of detrimental effects are presently unknown. clip-path: url(#SVGID_2_); If you have PKU, the severity of your condition would depend on what type of PKU you have. Without this enzyme, the body cant process this amino. Phenylalanine is an essential amino acid and it is found in nearly all foods which contain protein: meat (of all kinds), dairy products, nuts, beans, tofu the list goes on and on. Amino acids are the building blocks of protein. If we combine this information with your protected Phenylalanine can cause intellectual disabilities, brain damage, seizures and other problems in people with PKU. Phenylalanine is one of the building blocks (amino acids) of proteins. BioMarin Pharmaceuticals, Inc.; 2020. https://www.palynziq.com/. When they can't, it . Abnormality of Cardiovascular System Morphology. Do I need to take any vitamins or supplements? I personally know of several people who refuse to drink diet soda because the word "phenylketonurics" sounds too scary! include protected health information. Phenylketonuria (PKU) is a rare disorder you inherit from your parents. Mayo Clinic on Incontinence - Mayo Clinic Press, NEW The Essential Diabetes Book - Mayo Clinic Press, NEW Mayo Clinic on Hearing and Balance - Mayo Clinic Press, FREE Mayo Clinic Diet Assessment - Mayo Clinic Press, Mayo Clinic Health Letter - FREE book - Mayo Clinic Press, Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic School of Continuous Professional Development, Mayo Clinic School of Graduate Medical Education, Book: Mayo Clinic Family Health Book, 5th Edition, Newsletter: Mayo Clinic Health Letter Digital Edition. If babies are put on the low protein diet at a young age, and they follow the diet throughout life, there is no evidence that their PKU will be a major hindrance in their accomplishments. It may even help improve diagnosis and treatment of more common diseases. Women who have PKU and become pregnant are at risk of another form of the condition called maternal PKU. .st0 { You may have seen the warning on the label of a diet soda: Phenylketonurics contains phenylalanine? enable-background: new; I hope this helps! If you don't have PKU, you probably don't need to worry about harmful health effects of phenylalanine with certain important exceptions. PKU is caused by a change in the phenylalanine hydroxylase (PAH) gene. All rights reserved. Advertising on our site helps support our mission. It is a term that describes people who have PKU. This content does not have an English version. 1. Symptoms may start to appearas an Infant. Aspartame is an artificial sweetener made with phenylalanine. If phenylalanine is in it's natural form, then the answer to this question is a great big resounding NO. Since diagnosis and treatment of phenylketonuria (PKU) most often occur shortly after birth due to an abnormal newborn screen, noticeable symptoms are very rare. Because of this, use products with aspartame cautiously if you: If you aren't sure if phenylalanine or aspartame is a concern for you, talk to your doctor. Drugs. Phenylalanine is also sold as a dietary supplement. Based on the number of emails I have received from people like yourself, I believe that the effects of aspartame toxicity are more common than people realize. This medication replaces the enzyme that helps to break down phenylalanine, which doesnt work properly in PKU. High-intensity sweeteners. Phenylketonuria (fen-ul-keetone-YU-ree-ah, or PKU) is an inherited metabolic disorder in which the body cannot completely break down the protein (amino acid) phenylalanine. When babies are born in the United States, a heel stick blood test is done to test for various disorders - PKU is one of the disorders that is tested for. information submitted for this request. This material is provided for educational purposes only and is not intended for medical advice, diagnosis or treatment. Keep in mind that since children are rapidly growing, they already take in a much higher proportion of phenylalanine than adults do. The parents will notice the child being delayed in achieving their developmental milestones when compared to the childs siblings or else to other same aged children. The following are possible causes of PKU: A genetic mutation occurs when a gene becomes defective. Patient organizations are available to help find a specialist for this condition. Accessed Jan. 28, 2022. If phenylalanine is in it's natural form, then the answer to this question is a great big resounding NO. Phenylketonuria, commonly known as PKU, is a genetic disease. But, if this was not possible, such babies will develop signs and symptoms related to the disease in few months time. U.S. Food and Drug Administration. https://rarediseases.info.nih.gov/diseases/7383/phenylketonuria. Common symptoms of problems in the nervous system include trouble moving, speaking, swallowing, breathing, or learning. Braggs Coconut Aminos, All-Purpose Seasoning, Liquid Aminos Side Effects And How To Reverse It, How To Start All-Fruit Diet and How It Will Benefit You, Creatine Anti Aging Benefits + Skincare Products And Supplements To Try, Menopause And Anxiety: Whats The Connection, Pea Milk: Hows It Made And What It Taste Like. PKU is an inherited disorder that makes a person unable to digest the amino phenylalanine (Phe). Any use of this site constitutes your agreement to the Terms and Conditions and Privacy Policy linked below. This diet has been specifically designed for children diagnosed with PKU and should not be used for a child without PKU. If you are a Mayo Clinic patient, this could About & Disclaimer | Terms | Privacy | Contact, Amino Acids and their Structure and Function, How Proteins are Broken down into Amino Acids, What Does a Positive TB Test Look Like? One cup (240 ml) of black coffee contains about 3 calories and very small amounts of protein, fat, and trace . What is Phenylketonuria (PKU)? Babies born to women with high phenylalanine levels don't often inherit PKU. Inheritance is autosomal recessive manner. Milk, eggs, and other common foods contain phenylalanine. Phenylalanine is an amino acid naturally found in many proteins such as meat, milk and eggs. Phenylalanine occurs naturally in many protein-rich foods, such as milk, eggs and meat. The easiest way to lookup drug information, identify pills, check interactions and set up your own personal medication records. Nature Reviews Disease Primers. I hope this webpage has answered some of the questions you originally went searching for regarding the scary-sounding warning: Phenylketonurics - Contains Phenylalanine! Phenylketonuria (PKU) is a rare, autosomal recessive disease that prevents the body from breaking down one of the amino acids found in nearly all proteins: phenylalanine (Phe). Learn more about PKU in this article. As such, when they eat anything that contains Phe, This amino builds up in their body. Phenylalanine occurs naturally in many protein-rich foods, such as milk, eggs and meat. Most high protein foods contain much much more of the amino than a diet soda or other diet drink does. Because of this, use products with aspartame cautiously if you: If you aren't sure if phenylalanine or aspartame is a concern for you, talk to your doctor. Reference: Data from the Newborn Screening Codingand Terminology Guide is available here. Diet drinks and any other products which containaspartamemust bear the following health warning: Phenylketonurics: Contains Phenylalanine.. Depending on the severity of the PKU, babies who were not treated for the disorder and put on the special diet at birth run the risk of suffering irreversible mental retardation within the first year of life. We know the word sounds like a long, complex, and scary term. the unsubscribe link in the e-mail. https://www.nichd.nih.gov/health/topics/pku/conditioninfo/faqs. I want to note that I am NOT a medical professional (my Ph.D. is in Astronomy, not medicine), and you should never make important decisions about your health without the guidance of a medical specialist. So - kind of like how the word "diabetics" refers to people that have "diabetes", "phenylketonurics" refers to the people that have "phenylketonuria". These days, most doctors that provide care for PKU patients preach that the low protein PKU diet should be a way of life and, if we value our health and well-being, those of us with the disorder should never stray from it. If a person with uncontrolled PKU has high levels of phenylalanine during pregnancy, this can cause intellectual disability, birth defects and other problems in their baby, even if the baby doesn't have PKU. Phenylalanine isn't a health concern for most people. Zeratsky KA (expert opinion). I myself, am one of these phenylketonurics and the warning on diet soda cans is included merely to inform people like me that the product contains the synthetic chemical ASPARTAME . Most often, PKU is passed to children by two parents who are both carriers of the changed gene, but don't know it. The unwanted build-up of phenylalanine in the bodies of Phenylketonurics may be significantly worsened by the unknowing consumption of aspartame-sweetened products like diet coke. In the past, children were taken off the low protein PKU diet at age 5 or 6 because it was believed that at this age the brain had developed to the point where elevated levels of phenylpyruvic acid in the blood would not cause severe health problems. To provide you with the most relevant and helpful information, and understand which Is it OK to Eat Expired Canned Foods? When your body cant process the amino acids that you eat in your diet, they build up in your blood and tissues. Health Resources & Services Administration. Why Do We Say a Fifth of Whiskey, Rum, or Vodka? Rare disease umbrella organizations focus on improving the lives of all those impacted by rare diseases through education and advocacy efforts. I have seen the effects of aspartame toxicity and have discussed symptoms of aspartame poisoning with literally hundreds of people who have found this website. This gene helps create the enzyme needed to break down phenylalanine by change... 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