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