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Argininemia (ARG)

is a rare genetic disorder caused by the partial or complete lack of the enzyme Arginase.

Patients are prescribed a protein-restricted diet to prevent high levels of ammonia in the body.

This App is designed to support families and children with ARG treated with a protein-restricted diet.
What is ARG?
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Argininemia (ARG), also known as Arginase deficiency is a rare genetic disorder caused by the partial or complete lack of the enzyme Arginase. Read more on CPS-1 via this link.
Arginase controls the last reaction in the urea cycle and therefore ARG is part of a group of disorders known as the Urea Cycle Disorders (UCD).
Arginase removes nitrogen from the amino acid Arginine, a molecule made by earlier enzymes in the urea cycle.
When the enzyme Arginase is damaged or missing, people are not able to produce urea normally and nitrogen builds up in the blood in the form of ammonia.
Arginine is an important amino acid that makes up protein in foods. Protein is important for growth in children and health maintenance in adults.
In combination, high levels of ammonia and Arginine are believed to cause intellectual disorders as well as ARG symptoms.
In most cases symptoms of ARG are not presented the first few months to a year of life; infants are rarely found with severe hyperammonemia.
Untreated people with ARG may present, but are not limited to, symptoms such as stiffness in the legs (spasticity), decreased stature, intellectual delays, and seizures.
Why a diet?
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To minimize the build up of ammonia in the blood, people with ARG are prescribed a low protein diet and Arginine is carefully monitored.
People living with ARG are prescribed their daily protein restriction by their doctor or dietitian. These daily goals often need to be changed based on each person’s nutritional needs and age requirements.
Calculating and tracking protein intake can be a very hard and troublesome task for those with protein-restricted diets.
We hope this App makes daily protein counting and meal planning easier for people following low protein diets.
For whom?
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This App is designed to support families and children with ARG treated with a protein-restricted diet.
This App is not a substitute for the health care provided by your medical team!