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Inherited Metabolic Disorders

Some people are born with a genetic disorder that prevents the body from processing certain nutrients properly.

The term inherited metabolic disorder covers a wide range of conditions including disorders related to fat, carbohydrate and protein. Examples include PKU, HCU, MSUD, GA1, IVA and MMA/PA.

These hereditary disorders are most often diagnosed at birth, or early on in life. Defects with certain enzymes prevent the body from breaking down certain types of nutrients. If we can’t break down those nutrients, we can't get rid of certain chemicals, and they build up in the body over time. Without careful monitoring and dietary management, these chemicals can have various consequences, including delays in physical and mental development.

In the case of the amino acid disorder phenylketonuria (PKU), the condition stops patients from breaking down the essential amino acid phenylalanine, which we consume in the protein we eat as part of a normal diet. In PKU, phenylalanine builds up in the brain and can cause long-term health issues.


The term inherited metabolic disorder covers many different conditions

The Low Protein diet

People with protein disorders can’t break down certain amino acids. This means they have to stick carefully to a low-protein diet. Careful eating choices reduce exposure to the amino acids in food that cause problems.

It's important that patients also take nutritional supplements of the amino acids and other nutrients our bodies need to work properly. Healthcare professionals help patients achieve this important balance by prescribing specialist medical foods for special medical purposes.

Many patients with inherited metabolic disorders make a lifelong commitment to a strict low protein diet

Support for people with inherited metabolic disorders

Since the 1960s, we’ve been developing products to support many of our patients with inherited metabolic disorders. Our patients have lifelong conditions, and we're proud to be their partner for life. We hold events for patients, their families and carers, where we support them and they can meet and support each other – there’s a real sense of community. We provide low protein cookery classes to help families with mealtimes, and to show patients how to make the most of what they can eat. We have also developed a LowPro App to make tracking and managing the low protein diet simpler for patients.


Nutricia's metabolic product ranges are tailored to meet the needs of patients with inherited metabolic disorders


Visit our Resource Centre for a wide range of professional resources to support you and your patients.


Find out about Nutricia’s wide range of specialised nutritional products.


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Nutricia offers access to a wide range of support services and resources including:

  • • Our free Sampling Service to order product samples and have them sent to your patients' homes.
  • • A range of CPD accredited e-learning programmes.
  • • Order free paper copies of our support tools for yourself or to use with your patients.
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