Journal article

Dietary protein in urea cycle defects: How much? Which? How?

Avihu Boneh

Molecular Genetics and Metabolism | ACADEMIC PRESS INC ELSEVIER SCIENCE | Published : 2014


Dietary recommendations for patients with urea cycle disorders (UCDs) are designed to prevent metabolic decompensation (primarily hyperammonaemia), and to enable normal growth. They are based on the 'recommended daily intake' guidelines, on theoretical considerations and on local experience. A retrospective dietary review of 28 patients with UCDs in good metabolic control, at different ages, indicates that most patients can tolerate a natural protein intake that is compatible with metabolic stability and good growth. However, protein aversion presents a problem in many patients, leading to poor compliance with the prescribed daily protein intake. These patients are at risk of chronic protein..

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Funding Acknowledgements

I wish to acknowledge the dedicated work of the dietitians Dorothy Francis, Maureen Humphrey, Judy Nation, Jamie Errico and Jemma Watkins and the students Tatijana Gardeitchick, Simon Rodney and Julia Kuypers. This work was supported by the Victorian Government's Operational Infrastructure Support Program.