Growth & Metabolism / Inborn Errors of Metabolism

Dietary Management for Inborn Errors of Metabolism

Clinical symptoms of IEMs may appear at the very early stages of life. Dietary management, sometimes combined with pharmacological treatment, can help optimise health outcomes, making newborn screening essential. Once diagnosed, patients need to follow a restricted diet for the rest of their lives. As the nutrient they must eliminate is often essential for life, patients must complete their diet with specially adapted medical nutrition to fill the gap. Studies show that strict dietary control can significantly improve a patient’s health outcome.1, 2, 3, 4, 5

Once diagnosed, patients need to follow a restricted diet for the rest of their lives.

At Danone Nutricia Research, we continue to apply our expertise in metabolic disorders to develop dietary solutions for a wide variety of IEMs, meeting the needs of patients at every stage of their lives. This includes medical nutrition for inborn errors of: amino acid metabolism (e.g. phenylketonuria, Maple Syrup Urine Disease), carbohydrate metabolism (e.g. Galactosemia) and fatty acid metabolism (e.g. – very long-chain acyl-CoA dehydrogenase deficiency VLCAD).

We apply our expertise in metabolic disorders to develop dietary solutions for a wide variety of IEMs, meeting the needs of patients at every stage of their lives.

Through having a close and long-term proximity to people with errors of amino acid metabolism, we recognise the day to day challenges of managing a restricted diet. We have therefore developed a range of low-protein foods (e.g. flour and pasta) to support and facilitate energy intake for these patients.

View References

1.
Nardecchia F, et al. Mol Genet Metab, 2015;115(2-3):84-90.
2.
Huijbregts et al. J Inherit Metab Dis, 2002;25(6):419-30.
3.
Waisbren S. et al. Mol Genetnd Metabol, 2007;92:63-70.
4.
ten Hoedt, et al. J Inherit Metab Dis, 2011;34(1):165-71.
5.
Channon S, et al. Arch Dis Child, 2007;93(2):213-18.