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Arginine

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Product Range Arginine500 Arginine2000 Arginine5000
Pack Size 30 x 4g ℮ 30 x 4g ℮ 30 x 7.6g ℮
Ingredients Dried Glucose Syrup, L-arginine. Maltodextrin,
L-arginine.
L-arginine, Acidity regulator (E296),
Flavouring, Artificial sweetener
(Sucralose), Colour (E160a).



Description
Indication

Clinical Use

Urea Cycle Disorders1 (UCD)


UCD is an umbrella term encompassing six genetic disorders of protein metabolism, all of which result in impairment of the body’s ability to remove waste nitrogen. UCD can have acute, chronic and intermittent clinical manifestations occurring at any age. Their clinical hallmark is the hyperammonaemia crisis.


Due to impaired synthesis, L-arginine becomes an essential amino acid in all of the UCDs, with the exception of Arginase 1 deficiency. It must therefore be supplemented as either L-arginine or its precursor, L-citrulline.


Pyridoxine Dependent Epilepsy2 (PDE)


Pyridoxine-dependent epilepsy (PDE-ALDH7A1) is an autosomal recessive condition due to a deficiency of α-aminoadipic semialdehyde dehydrogenase, which is a key enzyme in L-lysine oxidation.


Although pyridoxine monotherapy is often effective in reducing seizure activity, most patients experience developmental delay and intellectual disability. Recent guidelines recommend ‘triple therapy’; pyridoxine hydrochloride, a lysine-restricted diet, and supplementing the diet with L-arginine.


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. Arginine5000 is now ready to serve. Water or permitted drinks should be consumed after Arginine5000.
For both Arginine500 and Arginine2000, wait 5 minutes*, 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

Arginine500, Arginine2000 & Arginine5000 can be used in conjunction with other Vitaflo products for the dietary management of UCD:

Further Reading


1. Häberle J, Burlina A, Chakrapani A, Dixon M, Karall D, Lindner M et al. Suggested guidelines for the diagnosis and management of urea cycle disorders: First revision. Journal of Inherited Metabolic Disease. 2019:42 (6); 1192-1230. DOI:10.1002/jimd.12100


2. Coughlin CR II, Tseng LA, Abdenur JE, Ashmore C, Boemers F, Bok LA, et al. Consensus guidelines for the diagnosis and management of pyridoxine-dependent epilepsy due to α-aminoadipic semialdehyde dehydrogenase deficiency. Journal of Inherited Metabolic Disease. 2021;44 (1):178–192. DOI: 10.1002/jimd.12332


Arginine500, Arginine2000 and Arginine5000 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.