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Cystine

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Product Range Cystine500
Pack Size 30 x 4g ℮
Ingredients Dried glucose syrup, L-cystine, Thickener (E415).


Description
Indication

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:

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

Cystine500 can be used in conjunction with other Vitaflo products for the dietary management of HCU:

Further Reading

 

1. Morris AA, Kožich V, Santra S, Andria G, Ben-Omran TI, Chakrapani AB, et al. Guidelines for the diagnosis and management of cystathionine beta-synthase deficiency. Journal of Inherited Metabolic Disease. 2017;40(1):49-74.

 

 

Cystine500 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.