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Leucine

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Product Range Leucine100
Pack Size 30 x 4g ℮
Ingredients Dried glucose syrup, L-leucine.


Description
Indication

Clinical Use

Leucine deficiency1


Many disorders of protein metabolism are managed through the restriction of natural protein. Such restrictions can increase the risk of essential amino acid deficiency, particularly if one or more of the following factors apply:


  • Tolerance for natural protein is low.
  • Due to feeding difficulties, lack of appetite or aversion, the patient consumes even less natural protein than recommended.
  • The biological value of the natural protein consumed is low.
  • Limited or no use of protein substitutes (for example, in some patients with organic acidaemias) or essential amino acid supplements (for example, in some patients with UCD).

Ongoing monitoring of plasma amino acids is required in patients following low protein diets as part of their dietary management. L-leucine is an essential amino acid; it should be supplemented in the case of an L-leucine deficiency, where other methods of increasing L-leucine intake (e.g. increasing natural protein) are not feasible.


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 and 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.




Further Reading


1. Dixon M, MacDonald A, White FJ. 28. Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Disorders. Clinical Paediatric Dietetics, 5th edition: Wiley Blackwell; 2020.


Leucine100 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.