 |
|
| Product Range | Cystine500 |
| Pack Size | 30 x 4g ℮ |
| Ingredients | Dried glucose syrup, L-cystine, Thickener (E415). |
Clinical Use
Homocystinuria (HCU)1
HCU, also known as cystathionine beta-synthase (CBS) deficiency, is a rare inherited disorder in the methionine catabolic pathway. Patients with severe HCU usually present in childhood with ectopia lentis, learning difficulties and skeletal abnormalities, whilst those with mild disease tend to present as adults with thromboembolism.
HCU results in raised levels of plasma methionine, homocysteine and other sulphur-containing metabolites, and low levels of plasma cysteine and cystathionine. Cysteine is an essential amino acid in HCU, and low concentrations may contribute to the pathogenesis.
Case reports suggest that cysteine deficiency can cause poor weight gain and growth, even in the presence of adequate energy intake. Protein substitutes used in the dietary management of HCU are enriched with L-cystine (a cysteine dimer), so additional supplementation is not usually necessary. In patients with severe deficiency, L-cystine may be added to the diet.
Preparation Guidelines
To be determined by the clinician or dietitian. Suggested methods include:
