View Product Catalogue

Search our database to view details of all our products.

More Details

View our Recipes

Click here to view our nutritional recipes

More Details

Latest news

CASE STUDY: Intensive Nutritional Intervention in a Frail, Elderly Patient

David Gray, Senior Specialist Dietitian at Guy’s and St Thomas’…

Case Study - Post-Pyloric feeding in gastroparesis: the role of high protein enteral feeds

Michelle Barry, Specialist Home Enteral Feeding Dietitian

There are several…

Available 1st March: 3 New Fortisip 2kcal Flavours

Help support your patients recovery with three tasty new flavours…

Nutricia Suggestion Engine - Conditions

Step 1

What information would you like to see?


Step 2

In order to show you information relevant to you, please select a condition of interest.

Step 3

Press the 'find' button below to find relevant articles,videos and podcasts.

You are viewing Consumer Information

Urea Cycle Disorders (UCD)

Urea cycle disorders are a group of inherited diseases characterised by incomplete ‘metabolism’ or processing of nitrogen. This metabolic fault causes ammonia to build up in the blood, which poisons the brain.

After we eat protein, our bodies break them down into amino acids. As part of this processing, nitrogen is generated as a waste product. A multi-step pathway of reactions known as the urea cycle is responsible for removing this nitrogen from the blood, turning it into urea and excreting it via the kidneys in urine. The steps of the pathway require enzymes and/or ‘co-factors’ (enzyme helpers) that perform a specific task in the overall processing of nitrogen to urea. In individuals with urea cycle disorders, one of the enzymes or an enzyme ‘co-factor’ from the pathway is absent or has reduced activity. The consequence of the deficiency is that the urea cycle produces ammonia instead of urea.

There are six different types of urea cycle disorders. Each is named after the deficient urea cycle enzyme or, in the case of NAGS, a deficient co-factor:

* arginase deficiency (ARG)
* argininosuccinate lyase deficiency (ASL)
* argininosuccinate synthetase deficiency (ASS)
* carbamyl phosphate synthetase deficiency (CPS)
* N-acetylglutamate synthase deficiency (NAGS)
* ornithine transcarbamylase deficiency (OTC)

Animal proteins are found in dairy products, meat, eggs and fish. Proteins are also found in plants including soy, legumes, grains and nuts. The body uses the amino acids from proteins to make its own proteins essential for life – for example enzymes; structural proteins in muscles, hair, skin, cells and cartilage; proteins that generate movement in muscles; or those involved in cell functioning or immune responses. Requirements are higher in childhood as proteins are needed to support growth and development. Certain amino acids must come from the diet, as the body cannot manufacture them itself. These are known as ‘essential’ amino acids. In periods of fasting or illness, the body often switches to use its own proteins, and stored fats, to generate energy.

HCP Information for Urea Cycle Disorders (UCD)

The content provided by Nutricia is for information purposes only and is in no way intended to be a substitute for medical consultation with your doctor, dietitian or healthcare professional. The information, opinions, and recommendations presented in these pages are not intended to replace the care of your own doctor, dietitian or healthcare professional. Before you make any changes in the management of your diet / treatment or any other persons diet /treatment you should always consult your doctor, dietitian or healthcare professional. Although we carefully review our content, Nutricia cannot guarantee or take responsibility for the medical accuracy of documents we publish, nor can Nutricia assume any liability for the content of any web site linked to our site. © 2016 Nutricia. All rights reserved.