Dietary patterns analysis in PKU

  • Research type

    Research Study

  • Full title

    Dietary patterns analysis in Phenylketonuria (PKU)

  • IRAS ID

    185896

  • Contact name

    Sharon Evans

  • Contact email

    evanss21@me.com

  • Sponsor organisation

    Birmingham Children's Hospital Research & Development

  • Duration of Study in the UK

    0 years, 6 months, 0 days

  • Research summary

    Phenylketonuria (PKU), a genetic condition causing inability to breakdown the protein phenylalanine, causes brain damage if untreated. A strict low-protein diet is essential. Recent reports suggest that overweight is more common in phenylketonuria (PKU), particularly girls, than in the general population. PKU is treated with a low protein diet supplemented with phenylalanine-free L-amino acids, food starches, sugars, fats, fruit and vegetables. Although much is known about what patients with PKU should eat, little is known about what they actually consume and how this relates to their nutritional status. No detailed dietary pattern analysis (DPA) and lifestyle studies have been published in PKU. DPA provides a broad picture of food and nutrient consumption, and may be more predictive of disease risk than individual foods or nutrients. Food frequency questionnaires (FFQ) are commonly used to collect data about food patterns.

    This study aims to develop the first validated self-administered FFQ specifically for use in PKU, based on permitted commonly eaten low-protein foods. PKU subjects aged 5-16y (n=50) and age, gender and ethnicity-matched control children (n=50) will complete the FFQ twice with a 4-week interval to check reproducibility. Results will be compared with 5 repeated 24-hour dietary recalls taken 7-10 days apart using the same participants to check accuracy.

    Once validated the FFQ will be used to study dietary patterns in PKU to tailor dietary advice. It is also planned to extend this work to other countries in Europe.

  • REC name

    London - Queen Square Research Ethics Committee

  • REC reference

    15/LO/1463

  • Date of REC Opinion

    1 Sep 2015

  • REC opinion

    Further Information Favourable Opinion