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Phenylalanine

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Phenylalanine box
Product Range Phenylalanine 50
Pack Size 30 x 4g ℮
Ingredients Dried glucose syrup, L-phenylalanine.


Description
Indication

Clinical Use

Hereditary tyrosinaemia type I (HT-1)1,2


HT-I is an autosomal recessive inherited disorder of the amino acid L-tyrosine. The main organs affected are the liver, kidneys and peripheral nervous system, with death in childhood common in untreated individuals.


The management of HT-I includes the dietary restriction of both L-tyrosine and its precursor amino acid, L-phenylalanine. The restriction of L-phenylalanine can lead to low plasma levels of this essential amino acid. Low L-phenylalanine concentrations may be detrimental to development. Supplementing the diet with enough L-phenylalanine to manage low levels, without leading to an excessive rise in L-tyrosine levels, is sometimes indicated.


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.
4. Wait 5 minutes*, shake again and serve.
(* Waiting time will enhance dispersion of the powder in the water.)




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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

Phenylalanine50can be used in conjunction with other Vitaflo products for the dietary management of HT-1:

Further Reading


1. de Laet C, Dionisi-Vici C, Leonard JV, McKiernan P, Mitchell G, Monti L et al. Recommendations for the management of tyrosinaemia type I. Orphanet Journal of Rare Diseases 2013: 8:8. DOI: 10.1038/gim.2017.101.


2. Chinsky JM, Singh R, Ficicioglu C, van Karnebeek CDM, Grompe M, Mitchell G et al. Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendations. Genetics in Medicine. 2017; 19(12): doi: 10.1038/gim.2017.101


Phenylalanine50 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.