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Phenylketonuria
Early diagnosis is key to treating PKU, a rare genetic disorder that results in an amino acid imbalance in the body. According to research or other evidence, the following self-care steps may be helpful.
- Fight back with fish oil
Take a daily supplement containing 500 mg for every 8.8 pounds of body weight to prevent deficiencies caused by the PKU diet
- Seek support from supplements
Prevent vitamin and mineral deficiencies caused by the PKU diet by taking supplements that provide the daily value of L-tyrosine, vitamin K, vitamin B12, and selenium
- Discover branched-chain amino acids
Under a healthcare provider’s supervision, improve mental functioning by adding amino acid supplements to each meal and at bedtime; take 150 mg per 2.2 pounds (1 kg) of body weight each of valine and isoleucine, and 200 mg per 2.2 pounds (1 kg) of body weight of leucine
- Monitor amino acids in your diet
Work with your healthcare provider and a nutritionist to maintain a phenylalanine-restricted diet that is nutritionally adequate
- Pass up aspartame
Help prevent increased phenylalanine intake by steering clear of soft drinks, candy, and other foods and drinks sweetened with aspartame (Nutrasweet)
Also indexed as:
- phenylketonuria,
- phenylketonuria (PKU),
- PKU
About this treatment
About This Condition
Phenylketonuria (PKU) is a rare genetic disorder that results in excessive accumulation of the amino acid, phenylalanine, and reduced levels of the amino acid, L-tyrosine, in the blood.
If untreated, high levels of phenylalanine can cause severe mental retardation, behavioral disturbances, and other brain and nerve problems. Fortunately, newborn screening programs now identify most cases of PKU in the United States and other countries. Early diagnosis and treatment is the key to reducing or preventing PKU-related conditions. Gene therapy is currently being researched as a possible cure. Research is also being conducted on methods to decrease levels of phenylalanine in the blood through the use of certain enzymes and amino acids.
Symptoms
Infants with PKU may be lethargic, feed poorly, and have a “mousy” odor from their sweat and urine. Eczema, sensitivity to sunlight, and light skin are also characteristic of PKU. Symptoms of children with untreated PKU include significantly diminished mental capacity, hyperactivity, and seizures.
Last Review: 05-11-2011
Copyright © 2011 Aisle7. All rights reserved. www.Aisle7.net
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The information presented in Aisle7 is for informational purposes only. It is based on scientific studies (human, animal, or in vitro), clinical experience, or traditional usage as cited in each article. The results reported may not necessarily occur in all individuals. Self-treatment is not recommended for life-threatening conditions that require medical treatment under a doctor's care. For many of the conditions discussed, treatment with prescription or over the counter medication is also available. Consult your doctor, practitioner, and/or pharmacist for any health problem and before using any supplements or before making any changes in prescribed medications. Information expires June 2011.
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