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Valine

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Product Range Valine50 Valine1000
Pack Size 30 x 4g ℮ 30 x 4g ℮
Ingredients Dried glucose syrup, L-valine. Dried glucose syrup, L-valine.


Description
Indication

Clinical Use

Maple syrup urine disease (MSUD)1,2


MSUD is an inherited metabolic disorder caused by branched-chain α-ketoacid dehydrogenase (BCKD) deficiency, resulting in the accumulation of branched chain amino acids (BCAAs) L-leucine, L-isoleucine and L-valine. MSUD varies considerably in disease severity, with clinical features including seizures, ketoacidosis, hypoglycaemia, apnoea, ataxia and coma. Elevated L-leucine levels are toxic and associated with abnormal brain morphology and cognitive impairment. This may impact on social and psychomotor function.


The mainstay of dietary management is the restriction of dietary BCAAs, especially L-leucine, and the use of BCAA-free protein substitutes (PS). To promote anabolism of L-leucine, when L-leucine blood concentrations are high, additional supplementation with L-valine may be required. In addition, patients on dietary management can develop a L-valine deficiency, which is absent from their PS. This can lead to a rash and can also involve the eye and gut epithelium. Supplementation of the diet with L-valine is used to manage this.


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 1 minute*, 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

Valine50 & Valine1000 can be used in conjunction with other Vitaflo products for the dietary management of MSUD:

Further Reading


1. Frazier DM, Allgeier C, Homer C, Marriage BJ, Ogata B, Rohr F, et al. Nutrition management guideline for maple syrup urine disease: an evidence-and consensus-based approach. Molecular Genetics and Metabolism. 2014;112(3):210-7.


2. Hollak CE, Lachmann R. Inherited Metabolic Disease in Adults: A Clinical Guide: Oxford University Press; 2016.


Valine50 and Valine1000 are Food for Special Medical Purposes for the dietary management of inborn errors of metabolism. For use under medical supervision. For healthcare professional use only.