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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). |
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:
