How Is A Person’S Health Affected By Phenylketonuria?

How does phenylalanine affect the body?

Phenylalanine can cause intellectual disabilities, brain damage, seizures and other problems in people with PKU .

Phenylalanine occurs naturally in many protein-rich foods, such as milk, eggs and meat.

Phenylalanine is also sold as a dietary supplement..

Why is phenylalanine toxic to the brain?

The high plasma phenylalanine concentrations increase phenylalanine entry into brain and restrict the entry of other large neutral amino acids. In the literature, emphasis has been on high brain phenylalanine as the pathological substrate that causes mental retardation.

What foods are high in phenylalanine?

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

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.)

Does phenylalanine raise blood sugar?

Conclusion: In summary, phenylalanine in an amount moderately greater than that in a large protein meal stimulates an increase in insulin and glucagon concentration. It markedly attenuates the glucose-induced rise in plasma glucose when ingested with glucose.

What can someone with PKU eat?

PKU DietMeat, eggs, and fish.Bread, noodles, and wheat products.Nuts, peas, and beans (and products made from these foods).Milk and cheese.Chocolate.

What are the benefits of phenylalanine?

Phenylalanine is used for depression, attention deficit-hyperactivity disorder (ADHD), Parkinson’s disease, chronic pain, osteoarthritis, rheumatoid arthritis, alcohol withdrawal symptoms, and a skin disease called vitiligo.

Why does gum contain phenylalanine?

The side effects of sugar-free gum can vary based upon their ingredients: The phenylalanine in aspartame can be dangerous for people who have an inherited disorder called phenylketonuria (PKU). … Because the action of chewing can cause jaw muscles to tighten, chewing any type of gum excessively may cause jaw pain.

Where is phenylalanine hydroxylase found in the body?

Liver cellsLiver cells contain an enzyme called phenylalanine hydroxylase, which can add this group and convert phenylalanine to tyrosine. Thus as long as this enzyme is functional and there is a reasonable supply of phenylalanine, tyrosine can be synthesized in your body and does not have to be included in the food that you eat.

Is PKU a disability?

In order to qualify for Social Security Disability benefits due to a diagnosis of phenylketonuria, you must be able to prove that your condition prevents you from performing any type of substantial gainful work activity.

How does phenylketonuria cause mental retardation?

Mutations in the PAH gene can cause phenylketonuria (PKU), a disorder that can change cells in the brain. The faulty protein allows dangerously high levels of phenylalanine to accumulate in the brain, poisoning the cells. If a person with PKU consumes too much phenylalanine, the build-up can cause mental retardation.

Is phenylalanine the same as aspartame?

The ingredients of aspartame are aspartic acid and phenylalanine. Both are naturally occurring amino acids. Aspartic acid is produced by your body, and phenylalanine is an essential amino acid that you get from food. When your body processes aspartame, part of it is broken down into methanol.

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 …

What is the life expectancy of someone with phenylketonuria?

PKU does not shorten life expectancy, with or without treatment. Newborn screening for PKU is required in all 50 states.

How does phenylketonuria affect the brain?

PKU affects the brain. When neurotransmitters are not made in the right amounts, the brain cannot function properly. High blood Phe levels can cause disruptions in neurotransmitters like serotonin and dopamine, which are important for mood, learning, memory, and motivation.

When should I take phenylalanine?

Phenylalanine should be taken between meals, because protein found in food may compete for uptake of phenylalanine into the brain, potentially reducing its effect. D-phenylalanine has been used with mixed results to treat chronic pain, including pain caused by rheumatoid arthritis.

Does phenylalanine raise blood pressure?

Phenylalanine can increase a chemical in the body called tyramine. Large amounts of tyramine can cause high blood pressure. But the body naturally breaks down tyramine to get rid of it. This usually prevents the tyramine from causing high blood pressure.

How does phenylketonuria affect the body?

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.

What is phenylalanine side effects?

Side effects may include:Heartburn.Fatigue.Nausea.Constipation.Dizziness.Headache.Anxiety and hypomania (a milder form of mania)Sedation.More items…•

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.

Can you outgrow PKU?

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