We are independent & ad-supported. We may earn a commission for purchases made through our links.

Advertiser Disclosure

Our website is an independent, advertising-supported platform. We provide our content free of charge to our readers, and to keep it that way, we rely on revenue generated through advertisements and affiliate partnerships. This means that when you click on certain links on our site and make a purchase, we may earn a commission. Learn more.

How We Make Money

We sustain our operations through affiliate commissions and advertising. If you click on an affiliate link and make a purchase, we may receive a commission from the merchant at no additional cost to you. We also display advertisements on our website, which help generate revenue to support our work and keep our content free for readers. Our editorial team operates independently from our advertising and affiliate partnerships to ensure that our content remains unbiased and focused on providing you with the best information and recommendations based on thorough research and honest evaluations. To remain transparent, we’ve provided a list of our current affiliate partners here.

What Is PKU?

Tricia Christensen
By
Updated May 17, 2024
Our promise to you
WiseGeek is dedicated to creating trustworthy, high-quality content that always prioritizes transparency, integrity, and inclusivity above all else. Our ensure that our content creation and review process includes rigorous fact-checking, evidence-based, and continual updates to ensure accuracy and reliability.

Our Promise to you

Founded in 2002, our company has been a trusted resource for readers seeking informative and engaging content. Our dedication to quality remains unwavering—and will never change. We follow a strict editorial policy, ensuring that our content is authored by highly qualified professionals and edited by subject matter experts. This guarantees that everything we publish is objective, accurate, and trustworthy.

Over the years, we've refined our approach to cover a wide range of topics, providing readers with reliable and practical advice to enhance their knowledge and skills. That's why millions of readers turn to us each year. Join us in celebrating the joy of learning, guided by standards you can trust.

Editorial Standards

At WiseGeek, we are committed to creating content that you can trust. Our editorial process is designed to ensure that every piece of content we publish is accurate, reliable, and informative.

Our team of experienced writers and editors follows a strict set of guidelines to ensure the highest quality content. We conduct thorough research, fact-check all information, and rely on credible sources to back up our claims. Our content is reviewed by subject matter experts to ensure accuracy and clarity.

We believe in transparency and maintain editorial independence from our advertisers. Our team does not receive direct compensation from advertisers, allowing us to create unbiased content that prioritizes your interests.

PKU or phenylketonuria is a condition that affects about 1 in 15,000 people. Those with this disorder lack the enzyme phenylalanine hydroxylase, which allows the body to turn ingested phenylalanine into the amino acid tyrosine. Tyrosine helps the body synthesize proteins, and where it is not present, the results can be quite devastating.

Newborns are routinely checked for PKU, because if they have the condition, which is inherited recessively, they must be on a special diet which is very low in phenylalanine. Phenylalanine unfortunately occurs in most proteins, like eggs, nuts, meat, and breast or dairy milk. Phenylalanine is also present in most infant formulas. If the child with the condition eats these foods, he or she will develop irreversible mental retardation, hence the mandatory testing.

The testing is fairly simple, though a bit hard to watch for new moms. The newborn’s heel is pricked, then a small amount of blood is squeezed out and sent to a lab for analysis. The lab results usually come back within a few days, since following dietary restrictions are vital for the newborn, and the child or adult with this disorder.

One will notice that many products clearly state whether or not they contain phenylalanines. You can see such labels on sodas, as well as on packaged nuts and cheese in most cases. As well as avoiding protein rich foods, most starch-laden foods like potatoes and pasta have to be avoided as well, so the diet is limited. To give the body the necessary protein it needs, protein formulas have been specifically developed for those with PKU so they can grow and develop normally.

When women with this condition become pregnant, the presence of PKU in the fetal environment is linked to birth defects of varied types. It is particularly important that pregnant women with this disorder seek the advice of a high-risk obstetrician. Most important is a strict adherence to a PKU diet during the pregnancy to prevent birth defects in the fetus.

Since the diet of the person affected by the disorder is restrictive, there are many companies that now manufacture PKU foods that are very low in or completely absent of phenylalanines. These products, like egg substitutes, ice cream substitutes, and various soups or spaghetti sauces, as well as low protein breads and pastas, can help the person with this condition have a relatively normal diet. However, the cost of such food can be expensive, particularly for the more fancy substitutions.

With the proper diet, the person with this condition can expect to live a long and healthy life. His or her food choices may be limited, but generally PKU will cause no other symptoms or result in complications as the person ages.

WiseGeek is dedicated to providing accurate and trustworthy information. We carefully select reputable sources and employ a rigorous fact-checking process to maintain the highest standards. To learn more about our commitment to accuracy, read our editorial process.
Tricia Christensen
By Tricia Christensen , Writer
With a Literature degree from Sonoma State University and years of experience as a WiseGeek contributor, Tricia Christensen is based in Northern California and brings a wealth of knowledge and passion to her writing. Her wide-ranging interests include reading, writing, medicine, art, film, history, politics, ethics, and religion, all of which she incorporates into her informative articles. Tricia is currently working on her first novel.

Discussion Comments

Tricia Christensen

Tricia Christensen

Writer

With a Literature degree from Sonoma State University and years of experience as a WiseGeek contributor, Tricia...
Learn more
WiseGeek, in your inbox

Our latest articles, guides, and more, delivered daily.

WiseGeek, in your inbox

Our latest articles, guides, and more, delivered daily.