Ornithine Transcarbamylase deficiency (OTC)

is a genetic disorder caused by a deficiency in the in Ornithine Transcarbamylase enzyme.

To minimize the build up of ammonia in the blood, people with OTC are prescribed a low protein diet and Cit is carefully monitored.

This App is designed to support families and children with OTC on protein restricted diets.

What is OTC?

Ornithine Transcarbamylase deficiency (OTC) is a genetic disorder caused by a deficiency in the Ornithine Transcarbamylase enzyme. Read more on NAGS via this link.

OTC is an X-linked disorder, as a result, it occurs more often in boys (one X chromosome) than in girls (two X chromosomes).

Ornithine Transcarbamylase is responsible for the break down of the molecules carbamoyl phosphate and ornithine (Orn) into citrulline (Cit) in the urea cycle. OTC is part of group of disorders called Urea Cycle Disorders (UCD).

Cit is an important amino acid that makes up protein in foods. Protein is important for growth in children and health maintenance in adults.

When the enzyme Ornithine Transcarbamylase is damaged or missing, people are not able to produce urea normally and nitrogen builds up in the blood in the form of ammonia.

Cit is supplemented for some people with OTC to allow for an alternate excretion route for ammonia.

Initial symptoms of OTC, which usually appear in the first few days of life, can include: lethargy, poor appetite, poor control of breathing rate or body temperature.

Why a diet?

To minimize the build up of ammonia in the blood, people with OTC are prescribed a low protein diet and Cit is carefully monitored.

People living with OTC 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?

This App is designed to support families and children with OTC on protein restricted diets.

This App is not a substitute for the health care provided by your medical team!