What is a pku diet

What foods should you avoid if you have PKU?

People with PKU must avoid foods that are high in protein like meat, fish, poultry, dairy, soy, legumes (dried beans) or nuts. Some fruits and vegetables are higher in protein than others.

What does it mean to have PKU?

Phenylketonuria (commonly known as PKU) is an inherited disorder that increases the levels of a substance called phenylalanine in the blood. Phenylalanine is a building block of proteins (an amino acid) that is obtained through the diet. It is found in all proteins and in some artificial sweeteners.

What is the life expectancy of a person with phenylketonuria?

About one in 15,000 babies is born with PKU in the United States. PKU leads to a build-up of the amino acid phenylalanine, which is toxic to the nervous system. Without treatment, PKU can cause intellectual disabilities. PKU does not shorten life expectancy, with or without treatment.

What happens when someone with PKU eat protein?

A dangerous buildup of phenylalanine can develop when a person with PKU eats protein-rich foods, such as milk, cheese, nuts or meat, and even grains such as bread and pasta, or eats aspartame, an artificial sweetener. This buildup of phenylalanine results in damage to nerve cells in the brain.

What foods are rich in phenylalanine?

Phenylalanine is found in most protein-containing foods such as milk, eggs, cheese, nuts, soybeans, chicken, beef, pork, beans and fish.

What does PKU smell like?

If PKU is untreated, or if foods containing phenylalanine are eaten, the breath, skin, ear wax, and urine may have a “mousy” or “musty” odor. This odor is due to a buildup of phenylalanine substances in the body.

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Who is most likely to get PKU?

In the United States, PKU is most common in people of European or Native American ancestry. It is much less common among people of African, Hispanic, or Asian ancestry.

Is PKU a disability?

Because the severity of PKU cases vary from individual to individual, however, a diagnosis of PKU in and of itself is not enough to qualify a person for Social disability benefits.

What part of the body does PKU affect?

Phenylketonuria (PKU) is a treatable disorder that affects the way the body processes protein. Children with PKU cannot use a part of the protein called phenylalanine. If left untreated, phenylalanine builds up in the bloodstream and causes brain damage.

Can you grow out of PKU?

A person with PKU does not outgrow it and must stay on the diet for life.

Can people with PKU drink alcohol?

Drinking and Having PKU

Alcohol changes your brain processing and interferes with your judgment. High blood phe levels + alcohol dramatically increases these effects. “It doesn’t take much”. Even one drink together with high blood phe levels can significantly impair your thinking.

Is PKU more common in males or females?

Each year 10,000 to 15,000 babies are born with the disease in the United States and Phenylketonuria occurs in both males and females of all ethnic backgrounds (although it is more common in individuals of Northern European and Native American heritage.)

How do PKU patients get protein?

The remaining protein, which includes the essential amount of phe the body needs for growth and functioning, comes from food. Foods such as red meat, chicken, fish, eggs, milk, yoghurt, cheese, nuts and legumes (e.g. lentils, chick peas, kidney beans) are too high in protein to include in a diet for a person with PKU.

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What happens when phenylalanine accumulates in the body?

Phenylalanine is an essential nutrient, but some individuals are born with a genetic disorder, phenylketonuria (PKU), that prevents them from metabolizing phenylalanine, and, if untreated, phenylalanine accumulates in the body, becomes converted into phenylpyruvate, and the individual usually develops seizures, brain …

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