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Tyrosine

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Product Range Tyrosine1000
Pack Size 30 x 4g ℮
Ingredients Dried Glucose Syrup, L-Tyrosine, Thickener(E415).


Description
Indication

Clinical Use

Phenylketonuria (PKU)1


PKU is an autosomal recessive inborn error of L-phenylalanine (Phe) metabolism, caused by deficiency in the enzyme phenylalanine hydroxylase (PAH), which converts Phe to tyrosine. PAH deficiency leads to accumulation of Phe in the blood and brain. Symptoms of untreated PKU include irreversible intellectual disability, microcephaly, motor deficits, seizures and developmental delay.


Dietary management of PKU involves a low Phe diet in combination with Phe-free protein substitutes (PS). As Phe is a precursor to L-tyrosine, L-tyrosine becomes an essential amino acid when Phe intake is restricted. L-tyrosine is important in the production of brain neurotransmitters, thyroxin and melanin skin pigments. As L-tyrosine is already added to Phe-free PS, additional L-tyrosine supplementation is not usually required in routine care.


Individuals who do not take the prescribed amount of PS may have an inadequate intake of L-tyrosine. It is recommended that supplemental L-tyrosine be provided if blood L-tyrosine concentrations are consistently below the normal range.


Preparation Guidelines

To be determined by the clinician or dietitian. Suggested methods include:

1. Measure out 50ml of water into a shaker.
2. Add one sachet.
3. SHAKE vigorously for 30 seconds and serve.




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1. Pour the liquid protein substitute into a shaker.
2. Add in the SDAA.
3. Shake for 30 seconds.
4. Serve.




1. Mix the dry SDAA and protein substitute powders together.
2. Follow the protein substitute preparation instructions. You may wish to use extra water.
3. Serve.




Combinations

Tyrosine1000can be used in conjunction with other Vitaflo products for the dietary management of PKU:

Further Reading


1. Genetic Metabolic Dietitians International (GMDI). PKU Nutrition Management Guidelines 2016. Available from: https://managementguidelines.net/guidelines.php/90/overview/0/0/PKU%20Nutrition%20Guidelines/Version%201.12/Overview


2. van Wegberg AMJ, MacDonald A, Ahring K, B langer-Quintana A, Blau N, Bosch AM, et al. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet Journal of Rare Diseases. 2017;12(1):1-56.


Tyrosine1000 is a Food for Special Medical Purposes for the dietary management of inborn errors of metabolism. For use under medical supervision. For healthcare professional use only.